Chłoniak waldenströma – Diagnostyka i diagnoza

Chłoniak waldenströma
Diagnostyka i diagnoza

Chłoniak Waldenströma (WM) to rzadki, indolentny nowotwór limfoproliferacyjny charakteryzujący się obecnością monoklonalnego białka IgM w surowicy oraz naciekiem limfoplazmocytowym w szpiku kostnym (≥10%). Diagnostyka opiera się na kompleksowej ocenie: morfologii krwi (często niedokrwistość, małopłytkowość), elektroforezie białek z immunofiksacją, ilościowym oznaczeniu IgM (podwyższone stężenia), badaniu lepkości osocza, stężeniu β2-mikroglobuliny oraz biopsji szpiku kostnego z immunofenotypowaniem (typowy fenotyp: IgM+, CD19+, CD20+, CD22+dim, CD25+, CD27+, brak CD5, CD10, CD23). Kluczowym markerem molekularnym jest mutacja MYD88 L265P, obecna u 90-97% pacjentów, która wspomaga różnicowanie WM od innych limfoproliferacji. Dodatkowo badania mutacji CXCR4 (30-40% pacjentów) i TP53 dostarczają informacji prognostycznych i terapeutycznych. Badania obrazowe (CT, PET-CT, USG) służą ocenie zaawansowania i wykluczeniu transformacji histologicznej.

Diagnostyka chłoniaka Waldenströma

Chłoniak Waldenströma (Waldenström macroglobulinemia, WM) to rzadki, powoli rozwijający się nowotwór krwi, który charakteryzuje się obecnością monoklonalnego białka IgM w surowicy oraz naciekiem limfoplazmocytowym szpiku kostnego. Diagnostyka tej choroby wymaga kompleksowego podejścia i zastosowania wielu metod, które pozwalają na postawienie właściwej diagnozy i odróżnienie jej od innych chorób limfoproliferacyjnych¹².

Badania krwi

Badania krwi stanowią pierwszy i podstawowy etap diagnostyki chłoniaka Waldenströma. Często choroba zostaje wykryta przypadkowo podczas rutynowych badań krwi wykonanych z innych powodów³⁴. Kluczowe badania laboratoryjne obejmują:

Morfologię krwi obwodowej z rozmazem – może wykazać niedokrwistość, małopłytkowość lub inne niedobory komórek krwi⁵
Elektroforezę białek surowicy z immunofiksacją – pozwala na wykrycie i identyfikację monoklonalnej immunoglobuliny M (IgM)⁶
Ilościowe oznaczenie immunoglobulin – w WM charakterystyczne jest podwyższone stężenie IgM⁷
Badanie wolnych łańcuchów lekkich w surowicy⁸
Określenie lepkości osocza – istotne w przypadku podejrzenia zespołu nadlepkości⁹
Badania biochemiczne – oceniające funkcję nerek i wątroby, które mogą być uszkodzone przez białko IgM¹⁰
Stężenie β2-mikroglobuliny – marker prognostyczny¹¹
Oznaczenie krioglobulin i zimnych aglutynin – istotne w diagnostyce powikłań WM¹²

Wykrycie monoklonalnego białka IgM w surowicy jest konieczne do rozpoznania WM, jednak samo w sobie nie jest wystarczające do postawienia diagnozy¹³¹⁴.

Biopsja szpiku kostnego

Biopsja szpiku kostnego jest badaniem niezbędnym do potwierdzenia diagnozy chłoniaka Waldenströma¹⁵¹⁶. Podczas tej procedury pobiera się fragment szpiku kostnego (zazwyczaj z kości biodrowej) przy użyciu specjalnej igły. Pobrany materiał jest następnie badany pod kątem:

Obecności nacieku komórek limfoplazmocytowych – według kryteriów Mayo Clinic, dla rozpoznania WM wymagane jest co najmniej 10% nacieku szpiku przez komórki limfoplazmocytowe¹⁷¹⁸
Wzoru nacieku – w WM charakterystyczny jest wzór międzybeleczkowy (intertrabecular)¹⁹
Fenotypu immunologicznego komórek nowotworowych – typowy fenotyp dla WM to powierzchniowa ekspresja IgM, CD19, CD20 oraz markery CD22+dim, CD25+, CD27+ przy jednoczesnym braku ekspresji CD5, CD10, CD23²⁰²¹

Komórki pobrane podczas biopsji szpiku kostnego są również analizowane pod kątem obecności specyficznych markerów genetycznych charakterystycznych dla WM²².

Badania genetyczne i molekularne

Badania genetyczne i molekularne stanowią obecnie nieodłączny element diagnostyki chłoniaka Waldenströma, pozwalając na bardziej precyzyjne rozpoznanie oraz dostarczając informacji prognostycznych i predykcyjnych²³.

Mutacja MYD88

Mutacja L265P w genie MYD88 jest wykrywana u ponad 90-97% pacjentów z chłoniakiem Waldenströma, co czyni ją istotnym markerem diagnostycznym²⁴²⁵. Oznaczenie statusu mutacji MYD88 jest obecnie uważane za kluczowe w procesie diagnostycznym WM, szczególnie w różnicowaniu z innymi chorobami limfoproliferacyjnymi o nakładających się cechach klinicznych²⁶.

Ośrodek Binga dla Makroglobulinemii Waldenströma w Dana-Farber Cancer Institute odkrył rolę mutacji MYD88 w WM i opracował test molekularny dla najczęstszej mutacji MYD88 (L265P)²⁷. Obecność tej mutacji sprzyja diagnozie WM, podczas gdy jej brak może sugerować inne schorzenia²⁸.

Inne badania genetyczne

Oprócz badania mutacji MYD88, w diagnostyce WM wykorzystuje się również inne testy genetyczne:

Badanie mutacji CXCR4 – obecna u około 30-40% pacjentów z WM, wpływa na obraz kliniczny choroby i odpowiedź na leczenie²⁹³⁰
Badanie mutacji TP53 – związana z gorszym rokowaniem i wpływa na decyzje terapeutyczne³¹
Cytogenetyka – poszukiwanie specyficznych aberracji chromosomowych³²

Badania molekularne powinny być wykonywane na materiału z szpiku kostnego przy użyciu czułych technik, takich jak ASqPCR (allele-specific quantitative PCR) lub dPCR (digital PCR)³³.

Badania obrazowe

Badania obrazowe nie są konieczne do postawienia diagnozy WM, ale mogą być pomocne w ocenie zaawansowania choroby i wykluczeniu innych schorzeń³⁴.

Podstawowe badania obrazowe

Do najczęściej wykonywanych badań obrazowych w diagnostyce chłoniaka Waldenströma należą:

Tomografia komputerowa (CT) klatki piersiowej, jamy brzusznej i miednicy – pozwala na ocenę ewentualnego powiększenia węzłów chłonnych, śledziony, wątroby lub innych organów³⁵³⁶
Pozytonowa tomografia emisyjna (PET) lub PET-CT – może wykazać zwiększoną aktywność metaboliczną w zajętych chorobą tkankach³⁷
Badanie ultrasonograficzne – pomocne w ocenie organów wewnętrznych³⁸
Badanie dna oka – może wykazać nieprawidłowości w naczyniach siatkówki związane z zespołem nadlepkości³⁹

W przypadku podejrzenia transformacji histologicznej do bardziej agresywnego chłoniaka, badanie PET-CT jest szczególnie wartościowe, gdyż może wskazać obszary o wysokiej wartości SUVmax (maksymalnej standaryzowanej wartości wychwytu), typowe dla bardziej agresywnych chłoniaków⁴⁰.

Badania dodatkowe w szczególnych przypadkach

W zależności od objawów klinicznych mogą być konieczne dodatkowe badania obrazowe:

Badanie MRI mózgu i rdzenia kręgowego – w przypadku podejrzenia zajęcia układu nerwowego (zespół Binga-Neela)⁴¹
Badania obrazowe w kierunku amyloidozy – jeśli występują objawy sugerujące odkładanie się amyloidu⁴²

Rozpoznanie różnicowe

Diagnostyka różnicowa chłoniaka Waldenströma wymaga odróżnienia go od innych chorób przebiegających z obecnością monoklonalnej IgM, co może być wyzwaniem diagnostycznym⁴³⁴⁴.

Rozróżnienie WM od MGUS IgM

Gammapatia monoklonalna o nieokreślonym znaczeniu z IgM (IgM MGUS) to stan przedchorobowy, który może poprzedzać rozwój WM. Różnice diagnostyczne obejmują:

Stężenie IgM – w MGUS zwykle poniżej 3 g/dl⁴⁵
Naciek szpiku kostnego – w MGUS poniżej 10% komórek limfoplazmocytowych⁴⁶
Brak objawów narządowych i limfadenopatii w MGUS⁴⁷

Ryzyko progresji z IgM MGUS do WM wynosi około 1,5% rocznie⁴⁸.

Rozróżnienie WM od innych chorób limfoproliferacyjnych

WM należy różnicować z innymi chorobami limfoproliferacyjnymi B-komórkowymi, które mogą produkować monoklonalne IgM⁴⁹:

Szpiczak mnogi IgM – odróżnia się poprzez inny wzór nacieku szpiku i inny fenotyp komórek nowotworowych⁵⁰
Chłoniak strefy brzeżnej – pomocne w różnicowaniu są badania molekularne (obecność mutacji MYD88)⁵¹
Przewlekła białaczka limfocytowa – ma charakterystyczny fenotyp immunologiczny (CD5+, CD23+)⁵²
Inne chłoniaki z małych limfocytów B – różnicowanie na podstawie fenotypu i badań molekularnych⁵³

W przypadkach, gdy obraz kliniczny i patologiczny nie dają jednoznacznej diagnozy, oznaczenie statusu mutacji MYD88 może być decydujące⁵⁴.

Symptomatyczny i bezobjawowy WM

W diagnostyce istotne jest także rozróżnienie między symptomatycznym i bezobjawowym (tlącym się) WM⁵⁵.

Tlący się WM (Smoldering WM)

Tlący się chłoniak Waldenströma (SWM) charakteryzuje się:

Obecnością monoklonalnej IgM i naciekiem szpiku kostnego typowym dla WM⁵⁶
Brakiem objawów klinicznych związanych z chorobą⁵⁷
Brakiem wskazań do natychmiastowego rozpoczęcia leczenia⁵⁸

Około 20% pacjentów z WM ma postać tlącą się w momencie diagnozy⁵⁹. Rozróżnienie między SWM a aktywnym WM jest istotne, ponieważ SWM nie wymaga leczenia, a pacjenci poddani aktywnemu nadzorowi wykazują przeżywalność porównywalną z populacją ogólną dopasowaną pod względem wieku i płci⁶⁰.

Wskazania do rozpoczęcia leczenia

Leczenie jest wskazane u pacjentów z objawowym WM, u których występują⁶¹:

Objawy wynikające z nacieku szpiku – niedokrwistość (Hb < 10 g/dl), małopłytkowość (< 100 000/μl)⁶²
Powiększenie narządów – limfadenopatia, hepatosplenomegalia⁶³
Neuropatia związana z IgM⁶⁴
Zespół nadlepkości⁶⁵
Krioglobulinemia objawowa⁶⁶
Choroba zimnych aglutynin⁶⁷
Amyloidoza⁶⁸

Systemy oceny prognostycznej

W przeciwieństwie do wielu innych nowotworów, WM nie ma standardowego systemu stadiowania⁶⁹⁷⁰. Niemniej jednak istnieją systemy prognostyczne, które pomagają w ocenie rokowania i podejmowaniu decyzji terapeutycznych.

Międzynarodowy System Prognostyczny dla WM

Międzynarodowy System Prognostyczny dla WM (IPSS-WM) jest zwalidowanym modelem często stosowanym w praktyce klinicznej⁷¹. Uwzględnia on następujące czynniki:

Wiek – pacjenci powyżej 65 roku życia mają gorsze rokowanie⁷²
Stężenie hemoglobiny – poniżej 11,5 g/dl jest niekorzystnym czynnikiem⁷³
Liczba płytek krwi – poniżej 100 000/μl wskazuje na gorsze rokowanie⁷⁴
Stężenie β2-mikroglobuliny – podwyższone wartości są niekorzystne⁷⁵
Stężenie monoklonalnej IgM – wysokie wartości mogą wskazywać na większą masę guza⁷⁶

Na podstawie tych czynników pacjenci są klasyfikowani do trzech grup ryzyka: niskiego, pośredniego i wysokiego, co pomaga lekarzom w wyborze leczenia i ocenie indywidualnego rokowania⁷⁷.

Dodatkowe czynniki prognostyczne

Oprócz czynników uwzględnionych w IPSS-WM, rokowanie u pacjentów z WM jest również związane z⁷⁸:

Podwyższonym stężeniem dehydrogenazy mleczanowej (LDH) – niekorzystny czynnik prognostyczny⁷⁹
Niskim stężeniem albuminy w surowicy – wiąże się z gorszym rokowaniem⁸⁰
Statusem mutacji genetycznych – MYD88, CXCR4 i TP53 mogą wpływać na rokowanie i odpowiedź na leczenie⁸¹

Te dodatkowe czynniki, wraz z oceną kliniczną i preferencjami pacjenta, pomagają w podejmowaniu indywidualnych decyzji dotyczących leczenia⁸².

Zalecenia diagnostyczne

Na podstawie aktualnych wytycznych można sformułować zalecenia dotyczące diagnostyki chłoniaka Waldenströma⁸³⁸⁴.

Schemat diagnostyczny

Kompleksowa diagnostyka WM powinna obejmować:

Badania krwi obwodowej:
- Morfologia z rozmazem
- Elektroforeza białek surowicy z immunofiksacją
- Ilościowe oznaczenie immunoglobulin (IgM, IgG, IgA)
- Badanie wolnych łańcuchów lekkich
- Ocena funkcji nerek i wątroby
- Stężenie β2-mikroglobuliny, LDH, albuminy
- Badanie lepkości osocza (w przypadku wysokiego stężenia IgM)
Biopsja szpiku kostnego:
- Ocena histopatologiczna
- Immunofenotypowanie komórek nowotworowych
- Badania molekularne (MYD88, CXCR4, TP53)
Badania obrazowe:
- CT klatki piersiowej, jamy brzusznej i miednicy
- W wybranych przypadkach – PET-CT lub inne badania obrazowe

W przypadku bezobjawowych pacjentów z niskim stężeniem monoklonalnej IgM (poniżej 1,5 g/dl lub 15 g/l) i prawidłowym stosunkiem wolnych łańcuchów lekkich, biopsja szpiku kostnego może nie być konieczna, jeśli nie ma objawów związanych z IgM⁸⁵. Jednak większość ekspertów zgadza się, że biopsja szpiku może dostarczyć istotnych informacji prognostycznych dotyczących ryzyka progresji, dlatego wskazania do jej wykonania powinny być rozważane indywidualnie⁸⁶.

Znaczenie diagnostyki w współczesnym leczeniu WM

Dokładna diagnostyka ma kluczowe znaczenie dla odpowiedniego leczenia pacjentów z WM⁸⁷:

Status mutacji MYD88 i CXCR4 wpływa na wybór terapii, szczególnie w kontekście stosowania inhibitorów kinazy tyrozynowej Brutona (BTK)⁸⁸
Przed rozpoczęciem leczenia ibrutyniebem należy sprawdzić status mutacji MYD88⁸⁹
Pacjenci z określonymi mutacjami mogą wymagać indywidualnego podejścia terapeutycznego⁹⁰

Właściwa diagnostyka pozwala na indywidualizację leczenia w oparciu o profil genetyczny, stan kliniczny pacjenta oraz jego preferencje, co może znacząco wpłynąć na skuteczność terapii i jakość życia⁹¹.

Podsumowanie diagnostyki chłoniaka Waldenströma

Chłoniak Waldenströma, jako rzadka choroba limfoproliferacyjna, wymaga kompleksowego podejścia diagnostycznego. Kluczowe elementy rozpoznania obejmują wykazanie obecności monoklonalnej IgM w surowicy oraz nacieku limfoplazmocytowego w szpiku kostnym⁹²⁹³.

Istotnym postępem w diagnostyce WM było odkrycie znaczenia mutacji MYD88 L265P, obecnej u ponad 90% pacjentów, która pomaga w różnicowaniu WM od innych chorób z gammapatią monoklonalną IgM⁹⁴.

Identyfikacja charakterystycznych cech klinicznych, laboratoryjnych, histopatologicznych i molekularnych pozwala na właściwe rozpoznanie i stratyfikację ryzyka, co ma kluczowe znaczenie dla wyboru optymalnego postępowania terapeutycznego⁹⁵.

Ze względu na rzadkość występowania i złożoność obrazu klinicznego WM, diagnostyka powinna być prowadzona w ośrodkach specjalizujących się w leczeniu tej choroby, co zwiększa szanse na właściwe rozpoznanie i optymalne leczenie⁹⁶.

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Materiały źródłowe

#1 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
The diagnosis of Waldenstrms macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. […] This, together with the increasing variety of tools and techniques available, makes it necessary to have a practical guidance for clinicians to perform the initial evaluation of patients with WM. […] We provide the procedures for multiparametric flow cytometry, fluorescence in situ hybridization and molecular tests, and with this offer guidance for a standardized diagnostic work-up and methodological workflow of patients with IgM monoclonal gammopathy of uncertain significance, asymptomatic and symptomatic WM. […] The Consensus Panel Recommendations from the Second International Workshop on Waldenstrms Macroglobulinemia (WM) state that the diagnosis of WM requires the following clinical and pathological criteria: presence of infiltration of clonal lymphoplasmacytoid cells documented by bone marrow (BM) biopsy (lymphoplasmacytic lymphoma (LPL)) and presence of monoclonal IgM in the serum, irrespective of the M-protein size.
#2 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
The diagnosis of Waldenstrms macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. […] This, together with the increasing variety of tools and techniques available, makes it necessary to have a practical guidance for clinicians to perform the initial evaluation of patients with WM. […] In this paper, we present the consensus recommendations and laboratory requirements for the diagnosis of WM developed by the European Consortium of Waldenstrms Macroglobulinemia (ECWM), for both clinical practice as well as the research/academical setting. […] The Consensus Panel Recommendations from the Second International Workshop on Waldenstrms Macroglobulinemia (WM) state that the diagnosis of WM requires the following clinical and pathological criteria: presence of infiltration of clonal lymphoplasmacytoid cells documented by bone marrow (BM) biopsy (lymphoplasmacytic lymphoma (LPL)) and presence of monoclonal IgM in the serum, irrespective of the M-protein size.
#3 How is WM Diagnosed? – International Waldenstromâs Macroglobulinemia Foundation
https://iwmf.com/how-is-wm-diagnosed/
Waldenstrom macroglobulinemia (WM) is often discovered incidentally when a person gets blood tests done for some other reason or during their annual check-up. Sometimes, people see their doctor complaining of specific symptoms or have a general sense of not feeling well. If signs and symptoms suggest that a person might have WM, a number of tests and exams will be performed to confirm, or rule out, a diagnosis of WM. […] Your doctor will start by getting a detailed medical history. He or she will ask questions about your past and current health, including: Any symptoms you are experiencing, particularly those that may be related to WM. […] Your doctor will order several blood tests to determine the types and amounts of antibodies in your blood. People with WM have a high level of the antibody IgM (immunoglobulin M).
#4 Tests for Waldenstrom Macroglobulinemia | American Cancer Society
https://www.cancer.org/cancer/types/waldenstrom-macroglobulinemia/detection-diagnosis-staging/how-diagnosed.html
Waldenstrom macroglobulinemia (WM) is often found when a person goes to see their doctor because of symptoms they are having, or because they just dont feel well and go in for a checkup. […] If signs or symptoms suggest that a person might have WM, exams and tests will be done to be sure. The most important tests will look for abnormal proteins in the blood and abnormal cells in the bone marrow. […] WM might be suspected if your doctor finds you have low blood cell counts or unusual protein levels on blood tests. […] Finding a monoclonal IgM antibody in the blood is needed to diagnose WM. […] The symptoms of WM and non-Hodgkin lymphoma (NHL) are not distinctive enough for a doctor to know for certain if a person has one of them, based on symptoms alone. […] This is the most important type of biopsy for WM, and is needed to confirm the diagnosis.
#5 Waldenstrom macroglobulinemia – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/waldenstrom-macroglobulinemia/diagnosis-treatment/drc-20359986
A physical exam, medical history and the following tests are used to diagnose Waldenstrom macroglobulinemia: […] Blood tests can show if there are too few healthy blood cells. Also, blood tests detect a protein made by the cancer cells. This protein is immunoglobulin M, which is also called IgM. […] Blood tests also can show how well organs are working. Results can show whether the IgM proteins are harming organs, such as the kidneys and the liver. […] During a bone marrow biopsy, a needle is used to take some bone marrow from the hipbone. The sample goes to a lab where it is tested for cancer cells. If there are cancer cells, more tests can give more information about the cells. […] Imaging tests can help show whether cancer has spread to other areas of the body. Imaging tests might include CT scans or positron emission tomography scans, which are also called PET scans.
#6 WaldenstrÃ¶m’s Macroglobulinemia Diagnosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancer-types/waldenstroms-macroglobulinemia/waldenstroms-macroglobulinemia-diagnosis.html
Early and accurate diagnosis of Waldenstrm’s macroglobulinemia helps you have the highest chance for successful treatment. However, the disease can be challenging to diagnose. It is important for a pathologist experienced in Waldenstrm’s to read your test results. […] If you have symptoms that may signal Waldenstrm’s, your doctor will examine you and ask you questions about your health and your family medical history. […] One or more of the following tests may be used to find out if you have Waldenstrm’s and if it has spread. These tests also may be used to find out if treatment is working. […] Blood and urine tests are used to determine and follow levels of abnormal proteins produced by Waldenstrm’s macroglobulinemia. In the blood, these proteins are called monoclonal proteins (M proteins) or paraproteins. They are measured by a test called serum protein electrophoresis (SPEP). In the urine, these proteins are known as Bence Jones proteins. They are measured by a urine protein electrophoresis (UPEP) on a 24-hour sample of urine. An additional test, called an immunofixation (IFE) may help find small traces of abnormal proteins in either the blood or urine. […] The pathologists at MD Anderson are highly specialized in diagnosing and staging Waldenstrm’s macroglobulinemia.
#7 WaldenstrÃ¶m macroglobulinemia: 2021 update on diagnosis, risk stratification, and management – PubMed
https://pubmed.ncbi.nlm.nih.gov/33368476/
Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients and is found in the majority of IgM MGUS patients. […] Waldenstrm macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.
#8 WaldenstrÃ¶m’s macroglobulinaemia – Symptoms, diagnosis and treatment | BMJ Best Practice
https://bestpractice.bmj.com/topics/en-gb/897
Waldenstrm’s macroglobulinaemia (WM) is a rare indolent B-cell lymphoma that most commonly occurs in older white men. […] Diagnosis is most often made following an incidental finding of an IgM monoclonal protein (paraprotein) on a blood test. […] WM (also referred to as lymphoplasmacytic lymphoma [LPL]) is an indolent B-cell lymphoma in which malignant lymphoplasmacytic cells infiltrate bone marrow and visceral organs, and hypersecrete an IgM monoclonal protein. […] Key diagnostic factors include age 70 years, male sex, and white ancestry. […] Other diagnostic factors include history of IgM monoclonal gammopathy of undetermined significance (MGUS), family history of B-cell lymphoproliferative disease, fatigue, weakness, shortness of breath, and peripheral neuropathy. […] 1st investigations to order include FBC with differential, haematinic test, peripheral blood smear, and serum protein electrophoresis with immunofixation. […] Investigations to consider include serum free light chains, serum viscosity, and lymph node biopsy.
#9 Macroglobulinemia – Hematology and Oncology – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/hematology-and-oncology/plasma-cell-disorders/macroglobulinemia
Plasma viscosity is measured to confirm suspected hyperviscosity. When hyperviscosity is present, plasma viscosity is usually 4.0 milliPascal-second (normal, 1.4 to 1.8 milliPascal-second). […] Do serum protein electrophoresis followed by serum and urine immunofixation and quantitative immunoglobulin levels.
#10 Waldenstrom macroglobulinemia – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/waldenstrom-macroglobulinemia/diagnosis-treatment/drc-20359986
A physical exam, medical history and the following tests are used to diagnose Waldenstrom macroglobulinemia: […] Blood tests can show if there are too few healthy blood cells. Also, blood tests detect a protein made by the cancer cells. This protein is immunoglobulin M, which is also called IgM. […] Blood tests also can show how well organs are working. Results can show whether the IgM proteins are harming organs, such as the kidneys and the liver. […] During a bone marrow biopsy, a needle is used to take some bone marrow from the hipbone. The sample goes to a lab where it is tested for cancer cells. If there are cancer cells, more tests can give more information about the cells. […] Imaging tests can help show whether cancer has spread to other areas of the body. Imaging tests might include CT scans or positron emission tomography scans, which are also called PET scans.
#11 Waldenstromâs macroglobulinemia: An overview
https://lymphomahub.com/medical-information/waldenstrom-s-macroglobulinemia-an-overview
To confirm diagnosis, there must be a presence of a monoclonal IgM paraprotein and a lymphoplasmacytic infiltrate in the bone marrow. WM can be diagnosed by: […] – Serum protein electrophoresis and immunofixation to detect IgM presence […] – Clonal infiltration of the bone marrow can be detected by positive expression of surface IgM, CD19, CD20, CD25, and CD27 […] – Bone marrow aspirate is also obtained to evaluate MYD88 and CXCR4 mutational status […] Additional diagnostics: […] – complete blood counts […] – comprehensive metabolic panel […] – immunoglobulins […] – -2 microglobulin […] – cryoglobulins […] – cold agglutinins […] – baseline computed tomography scans […] […] […] Guidance on diagnosis may vary between countries. Please read the section on key guidelines for further information.
#12 Waldenstromâs macroglobulinemia: An overview
https://lymphomahub.com/medical-information/waldenstrom-s-macroglobulinemia-an-overview
To confirm diagnosis, there must be a presence of a monoclonal IgM paraprotein and a lymphoplasmacytic infiltrate in the bone marrow. WM can be diagnosed by: […] – Serum protein electrophoresis and immunofixation to detect IgM presence […] – Clonal infiltration of the bone marrow can be detected by positive expression of surface IgM, CD19, CD20, CD25, and CD27 […] – Bone marrow aspirate is also obtained to evaluate MYD88 and CXCR4 mutational status […] Additional diagnostics: […] – complete blood counts […] – comprehensive metabolic panel […] – immunoglobulins […] – -2 microglobulin […] – cryoglobulins […] – cold agglutinins […] – baseline computed tomography scans […] […] […] Guidance on diagnosis may vary between countries. Please read the section on key guidelines for further information.
#13 Tests for Waldenstrom Macroglobulinemia | American Cancer Society
https://www.cancer.org/cancer/types/waldenstrom-macroglobulinemia/detection-diagnosis-staging/how-diagnosed.html
Waldenstrom macroglobulinemia (WM) is often found when a person goes to see their doctor because of symptoms they are having, or because they just dont feel well and go in for a checkup. […] If signs or symptoms suggest that a person might have WM, exams and tests will be done to be sure. The most important tests will look for abnormal proteins in the blood and abnormal cells in the bone marrow. […] WM might be suspected if your doctor finds you have low blood cell counts or unusual protein levels on blood tests. […] Finding a monoclonal IgM antibody in the blood is needed to diagnose WM. […] The symptoms of WM and non-Hodgkin lymphoma (NHL) are not distinctive enough for a doctor to know for certain if a person has one of them, based on symptoms alone. […] This is the most important type of biopsy for WM, and is needed to confirm the diagnosis.
#14 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
The diagnosis of Waldenstrms macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. […] This, together with the increasing variety of tools and techniques available, makes it necessary to have a practical guidance for clinicians to perform the initial evaluation of patients with WM. […] In this paper, we present the consensus recommendations and laboratory requirements for the diagnosis of WM developed by the European Consortium of Waldenstrms Macroglobulinemia (ECWM), for both clinical practice as well as the research/academical setting. […] The Consensus Panel Recommendations from the Second International Workshop on Waldenstrms Macroglobulinemia (WM) state that the diagnosis of WM requires the following clinical and pathological criteria: presence of infiltration of clonal lymphoplasmacytoid cells documented by bone marrow (BM) biopsy (lymphoplasmacytic lymphoma (LPL)) and presence of monoclonal IgM in the serum, irrespective of the M-protein size.
#15 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
Therefore, a BM biopsy remains mandatory for the differential diagnosis between WM, IgM-MGUS and other B-cell lymphoproliferative disorders (B-LPDs). […] Multiparametric flow cytometry (MFC) and molecular techniques may help to confirm the diagnosis, especially to discriminate WM from other IgM-secreting disorders. […] A progressive increase in the number of light-chain-isotype-positive B-cells from IgM-MGUS to smoldering and to symptomatic WM has been demonstrated. […] However, the pattern of antigen expression and the relative fractions of individual marker expressing clonal B-cells remain stable during disease progression. […] Asymptomatic patients with IgM monoclonal component below 1.5g/dl (or 15g/l) and normal serum free light-chain ratio have a very low-risk of progression to overt WM or other lymphoproliferative malignancies, and BM biopsy is not generally recommended at this stage, outside the context of clinical trials, and in the absence of any potential IgM-related symptom.
#16 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
At the Bing Center for Waldenstrm’s Macroglobulinemia at Dana-Farber Brigham Cancer Center, our specialists manage your diagnosis and treatment plan as a team. In our highly specialized center, your testing and care are coordinated from your first appointment. […] Because Waldenstrm’s macroglobulinemia (WM) is rare, it is sometimes misdiagnosed or mistreated. It can sometimes be mistaken for multiple myeloma or indolent lymphoma other B-cell malignancies. It’s important to be seen at or consult with a treatment center like ours that is experienced with this kind of cancer. […] The most accurate way to diagnose Waldenstrm’s is using a bone marrow biopsy supported by appropriate molecular testing for the MYD88 mutation. Our hematopathologists perform these tests every day and have deep experience in this area. The Bing Center lab discovered the role of the MYD88 mutation in WM and developed the molecular test for the most common MYD88 mutation (L265P).
#17 Tests for Waldenstrom Macroglobulinemia | American Cancer Society
https://www.cancer.org/cancer/types/waldenstrom-macroglobulinemia/detection-diagnosis-staging/how-diagnosed.html
For a diagnosis of WM, at least 10% of the cells in the bone marrow must be lymphoplasmacytoid lymphoma cells. […] WM is usually diagnosed with a bone marrow biopsy. […] In some lymphomas, the cells may have too many chromosomes, too few chromosomes, missing parts of chromosomes (called deletions), or other abnormalities. These changes can help identify the type of lymphoma.
#18 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/view/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] The rationale for using a higher cutoff of LPL infiltration (10%) to define WM by the Mayo Clinic criteria stems from the high prevalence of IgM MGUS and low rate of its progression to active WM (1.5%/year). […] The presence of the MYD88 mutation favors the diagnosis of WM.
#19 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
The quality and quantity of the material required for WM diagnosis are critical. […] However, WM diagnostic criteria still require a histological evaluation of the BM biopsy for the final diagnosis. […] Newer, patient-friendly molecular tools applicable to PB samples might be preferred over classical BM biopsy, but they are not yet sufficiently evaluated and standardized to provide a definitive diagnosis. […] A relatively large amount of BM is needed to perform (at least) MFC and molecular studies, and during aspiration of such a volume, there is a significant risk of hemodilution. […] The infiltration pattern is usually divided into 3 to 4 types, including nodular, para-trabecular, (interstitial), and diffuse. […] According to several reports, para-trabecular invasion pattern is one of the pathological features of WM and can be useful for differentiation from MZL.
#20 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
The characteristic immunophenotypic features of WM clonal B-cells are intracytoplasmic and surface light-chain restriction, as well as surface expression of pan-B-cell antigens (CD19, CD20), together with CD22+dim, CD25+, CD27+ and IgM+. […] Although WM has a non-specific immunophenotype, CD5, CD23, CD10, cyclin D1, LEF1, and CD56 staining is helpful in excluding most differential diagnoses. […] Detection of MYD88 and, more recently, CXCR4 somatic mutations in ctDNA from PB of WM patients is an area under development, with initial promising results, showing a high concordance with tumor burden.
#21 Waldenstrom Macroglobulinemia Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/207097-workup
Bone marrow aspiration and biopsy are required to establish the diagnosis of Waldenstrm macroglobulinemia. Bone marrow examination findings show infiltration by small lymphocytes showing plasmacytic differentiation. […] Flow cytometry results show B-cell features with surface expression of IgM and B-cell differentiation markers. Waldenstrm macroglobulinemia is characterized in most cases by a surface IgM+ sIgD+/- CD5- CD10- CD19+ CD20+ CD22+ CD23- CD25+ CD27+ CD75- CD79+ CD103- CD138- FMC7+ BCL- 2+ BCL- 6- PAX- 5+ immunophenotype. In practice, a sIgM+ CD5- CD10- CD19+ CD20+ CD23- immunophenotype in association with a nonparatrabecular pattern of infiltration is diagnostic of Waldenstrm macroglobulinemia. […] A number of mutations may be found in patients with Waldenstrm macroglobulinemia, including the following: The L265P mutation in MYD88 can be found in 93-97% of patients; compared with wild-type MYD88, it is associated with lower risk of disease transformation, longer survival, and better response to ibrutinib.
#22 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
How We Diagnose Waldenstrom’s Macroglobulinemia […] At the Bing Center for WaldenstrÃ¶m’s Macroglobulinemia at Dana-Farber Brigham Cancer Center), our specialists manage your diagnosis and treatment plan as a team. Because we are a highly specialized center, your testing and care are coordinated from your first appointment. […] Because WaldenstrÃ¶m’s is uncommon, it is sometimes misdiagnosed. It’s important to be seen at â or consult with â a treatment center like ours that is dedicated to this kind of cancer. […] Some patients are completely asymptomatic when they are diagnosed. WM is often suspected after a blood test shows increased IgM in symptomatic patients. […] WaldenstrÃ¶m’s can be mistaken for multiple myeloma or indolent lymphoma, both of which are also B-cell malignancies. […] The most accurate way to diagnose WaldenstrÃ¶m’s is by a bone marrow biopsy and supported by appropriate molecular testing for the MYD88 mutation. At our center, our hematopathologists perform these studies on a daily basis and we have deep experience in this area. The MYD88 mutation in WM was discovered in the Bing Center Laboratories, and molecular testing for the most common MYD88 mutation (L265P) was developed in our laboratory. […] We also perform genomic testing for CXCR4 and TP53 which impact treatment decisions. The CXCR4 mutation in WM was discovered in the Bing Center and its importance in impacting WM disease presentation and treatment outcomes was first reported in studies from the Bing Center. […] Your doctor may also conduct imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins.
#23 WaldenstrÃ¶m macroglobulinemia: diagnosis and treatment | Giannopoulos | Hematology in Clinical Practice
https://journals.viamedica.pl/hematology_in_clinical_practice/article/view/HCP.a2022.0014
Waldenstrm macroglobulinemia (WM), according to the 2017 World Health Organization classification, is defined as the co-occurrence of lymphoplasmacytic lymphoma involving the bone marrow with monoclonal gammopathy of the IgM class regardless of the concentration of monoclonal protein. […] Diagnostic characteristics in WM have changed significantly with the discovery of two molecular markers: MYD88 and CXCR4. […] The mutational status of these markers both affects clinical presentation and has shown therapeutic implications. […] Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia. […] The WHIM-like CXCR4(S338X) somatic mutation activates AKT and ERK, and promotes resistance to ibrutinib and other agents used in the treatment of Waldenstrom’s Macroglobulinemia. […] Gertz MA. Waldenstrm macroglobulinemia: 2019 update on diagnosis, risk stratification, and management. […] Kastritis E, Leblond V, Dimopoulos MA, et al. ESMO Guidelines Committee. Waldenstrm’s macroglobulinaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.
#24 Waldenstrom Macroglobulinemia Workup: Approach Considerations, Laboratory Studies, Imaging Studies
https://emedicine.medscape.com/article/207097-workup
Bone marrow aspiration and biopsy are required to establish the diagnosis of Waldenstrm macroglobulinemia. Bone marrow examination findings show infiltration by small lymphocytes showing plasmacytic differentiation. […] Flow cytometry results show B-cell features with surface expression of IgM and B-cell differentiation markers. Waldenstrm macroglobulinemia is characterized in most cases by a surface IgM+ sIgD+/- CD5- CD10- CD19+ CD20+ CD22+ CD23- CD25+ CD27+ CD75- CD79+ CD103- CD138- FMC7+ BCL- 2+ BCL- 6- PAX- 5+ immunophenotype. In practice, a sIgM+ CD5- CD10- CD19+ CD20+ CD23- immunophenotype in association with a nonparatrabecular pattern of infiltration is diagnostic of Waldenstrm macroglobulinemia. […] A number of mutations may be found in patients with Waldenstrm macroglobulinemia, including the following: The L265P mutation in MYD88 can be found in 93-97% of patients; compared with wild-type MYD88, it is associated with lower risk of disease transformation, longer survival, and better response to ibrutinib.
#25 WaldenstrÃ¶m macroglobulinemia: 2021 update on diagnosis, risk stratification, and management – PubMed
https://pubmed.ncbi.nlm.nih.gov/33368476/
Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients and is found in the majority of IgM MGUS patients. […] Waldenstrm macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.
#26 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
Therefore, a BM biopsy remains mandatory for the differential diagnosis between WM, IgM-MGUS and other B-cell lymphoproliferative disorders (B-LPDs). […] Multiparametric flow cytometry (MFC) and molecular techniques may help to confirm the diagnosis, especially to discriminate WM from other IgM-secreting disorders. […] MYD88L265P assessment is considered crucial to discriminate between WM and other B-LPDs with overlapping clinical features. […] Therefore, most groups currently agree that it may provide prognostic information about the risk of progression and the indication of the BM biopsy should be discussed. […] We aim to provide consensus recommendations of the European Consortium of Waldenstrms Macroglobulinemia (ECWM) on diagnostics in this lymphoma subtype. […] The work-up of suspected newly diagnosed WM patients should include pathological, MFC, and molecular studies guided by the recommendations shown in Table 4.
#27 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
At the Bing Center for Waldenstrm’s Macroglobulinemia at Dana-Farber Brigham Cancer Center, our specialists manage your diagnosis and treatment plan as a team. In our highly specialized center, your testing and care are coordinated from your first appointment. […] Because Waldenstrm’s macroglobulinemia (WM) is rare, it is sometimes misdiagnosed or mistreated. It can sometimes be mistaken for multiple myeloma or indolent lymphoma other B-cell malignancies. It’s important to be seen at or consult with a treatment center like ours that is experienced with this kind of cancer. […] The most accurate way to diagnose Waldenstrm’s is using a bone marrow biopsy supported by appropriate molecular testing for the MYD88 mutation. Our hematopathologists perform these tests every day and have deep experience in this area. The Bing Center lab discovered the role of the MYD88 mutation in WM and developed the molecular test for the most common MYD88 mutation (L265P).
#28 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
The International Prognostic Staging System for WM (IPSS-WM) is a validated model, often used in clinical practice, but needs to be reexamined in the current era. […] The prognosis of patients with WM is known to be impacted by certain clinical and laboratory features at initial presentation, with advanced age, elevated serum lactate dehydrogenase, and low serum albumin unfavorably affecting the outcome. […] The presence of the MYD88 mutation favors the diagnosis of WM.
#29 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
How We Diagnose Waldenstrom’s Macroglobulinemia […] At the Bing Center for WaldenstrÃ¶m’s Macroglobulinemia at Dana-Farber Brigham Cancer Center), our specialists manage your diagnosis and treatment plan as a team. Because we are a highly specialized center, your testing and care are coordinated from your first appointment. […] Because WaldenstrÃ¶m’s is uncommon, it is sometimes misdiagnosed. It’s important to be seen at â or consult with â a treatment center like ours that is dedicated to this kind of cancer. […] Some patients are completely asymptomatic when they are diagnosed. WM is often suspected after a blood test shows increased IgM in symptomatic patients. […] WaldenstrÃ¶m’s can be mistaken for multiple myeloma or indolent lymphoma, both of which are also B-cell malignancies. […] The most accurate way to diagnose WaldenstrÃ¶m’s is by a bone marrow biopsy and supported by appropriate molecular testing for the MYD88 mutation. At our center, our hematopathologists perform these studies on a daily basis and we have deep experience in this area. The MYD88 mutation in WM was discovered in the Bing Center Laboratories, and molecular testing for the most common MYD88 mutation (L265P) was developed in our laboratory. […] We also perform genomic testing for CXCR4 and TP53 which impact treatment decisions. The CXCR4 mutation in WM was discovered in the Bing Center and its importance in impacting WM disease presentation and treatment outcomes was first reported in studies from the Bing Center. […] Your doctor may also conduct imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins.
#30 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
We also perform genetic testing for CXCR4 and TP53 mutations, since these mutations impact treatment decisions. The Bing Center discovered the CXCR4 mutation in WM, as well as its importance in impacting WM disease presentation and treatment outcomes. […] Your doctor may also do imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins. […] Because Waldenstrm’s involves the bone marrow, it is considered to be a whole body disease. This means that there is no staging process. Still, certain diagnostic factors, such as age, levels of IgM, hemoglobin, presence of MYD88, CXCR4, and TP53 mutations in WM cells, and platelets can help inform expected outcomes. […] Waldenstrm’s can be difficult to diagnose and properly treat. Reasons to consider a second opinion include: To confirm your diagnosis. If you have received a diagnosis and want to be treated at Dana-Farber Brigham Cancer Center. To determine the optimal therapy, and whether any is needed at this time. To learn more about your cancer from specialists who are world leaders in this disease, and who have treated hundreds of other patients like you. To learn if you’re eligible for a clinical trial.
#31 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
How We Diagnose Waldenstrom’s Macroglobulinemia […] At the Bing Center for WaldenstrÃ¶m’s Macroglobulinemia at Dana-Farber Brigham Cancer Center), our specialists manage your diagnosis and treatment plan as a team. Because we are a highly specialized center, your testing and care are coordinated from your first appointment. […] Because WaldenstrÃ¶m’s is uncommon, it is sometimes misdiagnosed. It’s important to be seen at â or consult with â a treatment center like ours that is dedicated to this kind of cancer. […] Some patients are completely asymptomatic when they are diagnosed. WM is often suspected after a blood test shows increased IgM in symptomatic patients. […] WaldenstrÃ¶m’s can be mistaken for multiple myeloma or indolent lymphoma, both of which are also B-cell malignancies. […] The most accurate way to diagnose WaldenstrÃ¶m’s is by a bone marrow biopsy and supported by appropriate molecular testing for the MYD88 mutation. At our center, our hematopathologists perform these studies on a daily basis and we have deep experience in this area. The MYD88 mutation in WM was discovered in the Bing Center Laboratories, and molecular testing for the most common MYD88 mutation (L265P) was developed in our laboratory. […] We also perform genomic testing for CXCR4 and TP53 which impact treatment decisions. The CXCR4 mutation in WM was discovered in the Bing Center and its importance in impacting WM disease presentation and treatment outcomes was first reported in studies from the Bing Center. […] Your doctor may also conduct imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins.
#32 WaldenstrÃ¶m macroglobulinaemia (WM) symptoms and diagnosis | Blood Cancer UK
https://bloodcancer.org.uk/understanding-blood-cancer/lymphoma/waldenstrom-macroglobulinaemia/wm-symptoms-diagnosis/
To find out if you have WM, you may need to have blood tests, urine tests, CT scans, a bone marrow biopsy, and cytogenetic testing of blood or bone marrow samples. […] If your blood test results arent in the normal range, you may be asked to have a bone marrow biopsy. For this test, youll have a minor procedure where a small sample of bone marrow will be taken from part of your hip bone (your pelvis). This is sent to a laboratory to check for LPL cells. If these cells are found, its a strong sign you have WM. […] Your doctors may test for mutations in your genes to help diagnose your condition. This is called cytogenetic testing and it can be done using a blood sample.
#33 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
BM biopsy is mandatory for a correct diagnosis to distinguish between the different forms of IgM monoclonal gammopathies, and it is particularly informative in distinguishing between WM and MYD88L265P MZL cases with IgM monoclonal protein. […] MYD88L265P assessment is mandatory during the diagnostic work-up of WM and related disorders, and must be done in BM samples, using ASqPCR or dPCR.
#34 How is WM Diagnosed? – International Waldenstromâs Macroglobulinemia Foundation
https://iwmf.com/how-is-wm-diagnosed/
Since the symptoms of WM can be similar to those caused by other diseases or infections, a diagnosis of WM can only be confirmed by testing your bone marrow. […] Imaging tests use x-rays, sound waves, magnetic fields, or radioactive particles to produce detailed pictures of the inside of your body. They are not needed to diagnose WM; however, they help your doctor determine whether the cancer has spread beyond your bone marrow to other parts of your body.
#35 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
We also perform genetic testing for CXCR4 and TP53 mutations, since these mutations impact treatment decisions. The Bing Center discovered the CXCR4 mutation in WM, as well as its importance in impacting WM disease presentation and treatment outcomes. […] Your doctor may also do imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins. […] Because Waldenstrm’s involves the bone marrow, it is considered to be a whole body disease. This means that there is no staging process. Still, certain diagnostic factors, such as age, levels of IgM, hemoglobin, presence of MYD88, CXCR4, and TP53 mutations in WM cells, and platelets can help inform expected outcomes. […] Waldenstrm’s can be difficult to diagnose and properly treat. Reasons to consider a second opinion include: To confirm your diagnosis. If you have received a diagnosis and want to be treated at Dana-Farber Brigham Cancer Center. To determine the optimal therapy, and whether any is needed at this time. To learn more about your cancer from specialists who are world leaders in this disease, and who have treated hundreds of other patients like you. To learn if you’re eligible for a clinical trial.
#36 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
How We Diagnose Waldenstrom’s Macroglobulinemia […] At the Bing Center for WaldenstrÃ¶m’s Macroglobulinemia at Dana-Farber Brigham Cancer Center), our specialists manage your diagnosis and treatment plan as a team. Because we are a highly specialized center, your testing and care are coordinated from your first appointment. […] Because WaldenstrÃ¶m’s is uncommon, it is sometimes misdiagnosed. It’s important to be seen at â or consult with â a treatment center like ours that is dedicated to this kind of cancer. […] Some patients are completely asymptomatic when they are diagnosed. WM is often suspected after a blood test shows increased IgM in symptomatic patients. […] WaldenstrÃ¶m’s can be mistaken for multiple myeloma or indolent lymphoma, both of which are also B-cell malignancies. […] The most accurate way to diagnose WaldenstrÃ¶m’s is by a bone marrow biopsy and supported by appropriate molecular testing for the MYD88 mutation. At our center, our hematopathologists perform these studies on a daily basis and we have deep experience in this area. The MYD88 mutation in WM was discovered in the Bing Center Laboratories, and molecular testing for the most common MYD88 mutation (L265P) was developed in our laboratory. […] We also perform genomic testing for CXCR4 and TP53 which impact treatment decisions. The CXCR4 mutation in WM was discovered in the Bing Center and its importance in impacting WM disease presentation and treatment outcomes was first reported in studies from the Bing Center. […] Your doctor may also conduct imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins.
#37 Waldenstrom macroglobulinemia – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/waldenstrom-macroglobulinemia/diagnosis-treatment/drc-20359986
A physical exam, medical history and the following tests are used to diagnose Waldenstrom macroglobulinemia: […] Blood tests can show if there are too few healthy blood cells. Also, blood tests detect a protein made by the cancer cells. This protein is immunoglobulin M, which is also called IgM. […] Blood tests also can show how well organs are working. Results can show whether the IgM proteins are harming organs, such as the kidneys and the liver. […] During a bone marrow biopsy, a needle is used to take some bone marrow from the hipbone. The sample goes to a lab where it is tested for cancer cells. If there are cancer cells, more tests can give more information about the cells. […] Imaging tests can help show whether cancer has spread to other areas of the body. Imaging tests might include CT scans or positron emission tomography scans, which are also called PET scans.
#38 Facts About WaldenstrÃ¶m Macroglobulinemia | Fred Hutchinson Cancer Center
https://www.fredhutch.org/en/diseases/waldenstrom-macroglobulinemia/facts-resources.html
If your physician suspects you may have WM, they will do a physical exam to look for signs of the disease and ask about your symptoms, medical history, family history and risk factors. […] You might also have any or all of these tests: […] Blood tests to look for anemia and other complications of WM, to check your IgM levels (always elevated in WM) and to check the thickness of your blood (serum viscosity). […] Urine tests to look for abnormal proteins. […] Bone marrow tests taking samples of bone marrow and a small piece of bone from your pelvis using a needle (bone marrow aspiration and biopsy) to check for cancer cells. […] Lymph node biopsy taking samples from a lymph node to check for cancer cells. […] Molecular tests to help diagnose and identify specific features of your disease as well as inform your care. Genetic changes, like mutations in the MYD88 or CXCR4 gene, help predict whether you will develop WM symptoms and need treatment. […] Imaging tests such as a chest X-ray, ultrasound, computed tomography (CT) scan or positron emission tomography (PET) scan to see pictures of the inside of your body and look for enlarged lymph nodes, tumors or other cancer activity.
#39 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
How We Diagnose Waldenstrom’s Macroglobulinemia […] At the Bing Center for WaldenstrÃ¶m’s Macroglobulinemia at Dana-Farber Brigham Cancer Center), our specialists manage your diagnosis and treatment plan as a team. Because we are a highly specialized center, your testing and care are coordinated from your first appointment. […] Because WaldenstrÃ¶m’s is uncommon, it is sometimes misdiagnosed. It’s important to be seen at â or consult with â a treatment center like ours that is dedicated to this kind of cancer. […] Some patients are completely asymptomatic when they are diagnosed. WM is often suspected after a blood test shows increased IgM in symptomatic patients. […] WaldenstrÃ¶m’s can be mistaken for multiple myeloma or indolent lymphoma, both of which are also B-cell malignancies. […] The most accurate way to diagnose WaldenstrÃ¶m’s is by a bone marrow biopsy and supported by appropriate molecular testing for the MYD88 mutation. At our center, our hematopathologists perform these studies on a daily basis and we have deep experience in this area. The MYD88 mutation in WM was discovered in the Bing Center Laboratories, and molecular testing for the most common MYD88 mutation (L265P) was developed in our laboratory. […] We also perform genomic testing for CXCR4 and TP53 which impact treatment decisions. The CXCR4 mutation in WM was discovered in the Bing Center and its importance in impacting WM disease presentation and treatment outcomes was first reported in studies from the Bing Center. […] Your doctor may also conduct imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins.
#40 Transformed WaldenstrÃ¶m Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment
https://www.mdpi.com/2673-6357/3/4/44
The choice of the site of biopsy may be dictated by the 18 fluorodeoxyglucose-positron emission tomography (18FDG-PET/CT) results. […] The transformed sites are expected to have a high maximum standardized uptake value (SUVmax), similar to that observed in DLBCL. […] The presence of MYD88 L265P mutation has also been associated with a higher rate of CNS relapse in transformed WM. […] A tissue biopsy is mandatory to diagnose HT and may be dictated by the findings of an 18FDG-PET/CT scan, which is recommended for all patients suspected to have HT.
#41 Pathophysiology and Treatments of Complications of WaldenstrÃ¶m’s Macroglobulinemia | Published in Clinical Hematology International
https://chi.scholasticahq.com/article/124268-pathophysiology-and-treatments-of-complications-of-waldenstrom-s-macroglobulinemia
The gold standard for diagnosis of BNS is a stereotactic brain biopsy, the less invasive approach of identifying LPCs in the CSF and MRI findings with periventricular or leptomeningeal enhancement are adequate for diagnosis. […] A normal serum viscosity can help differentiate between the two. […] The treatment goal of HS includes rapidly reducing IgM levels to reduce serum viscosity, increase blood flow, and subsequently reduce symptoms and organ damage. […] The treatment algorithm for HS begins by assessing the severity of symptoms. […] Treatment of neurological complications of WM vary depending on the specific complication and its mechanism. […] There is no standardized treatment for asymptomatic patients, and it is unclear whether those with radiologic or CSF evidence of BNS should be treated.
#42 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#43 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
At the Bing Center for Waldenstrm’s Macroglobulinemia at Dana-Farber Brigham Cancer Center, our specialists manage your diagnosis and treatment plan as a team. In our highly specialized center, your testing and care are coordinated from your first appointment. […] Because Waldenstrm’s macroglobulinemia (WM) is rare, it is sometimes misdiagnosed or mistreated. It can sometimes be mistaken for multiple myeloma or indolent lymphoma other B-cell malignancies. It’s important to be seen at or consult with a treatment center like ours that is experienced with this kind of cancer. […] The most accurate way to diagnose Waldenstrm’s is using a bone marrow biopsy supported by appropriate molecular testing for the MYD88 mutation. Our hematopathologists perform these tests every day and have deep experience in this area. The Bing Center lab discovered the role of the MYD88 mutation in WM and developed the molecular test for the most common MYD88 mutation (L265P).
#44 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
Waldenstrms Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and 10% bone marrow involvement with lymphoplasmacytic cells. […] Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. […] Laboratory workup of suspected Waldenstrm Macroglobulinemia (WM) is enumerated in Table 1. […] Any mature B cell or plasma cell malignancy can potentially produce monoclonal IgM and hence, careful consideration of the differential diagnosis is vital. […] IgM MGUS is defined by presence of serum IgM concentration 3.0 g/dL, absence of lymphadenopathy, end organ damage and 10% marrow infiltration. It is important to do the confirmatory bone marrow biopsy to differentiate IgM MGUS from WM.
#45 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
Waldenstrms Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and 10% bone marrow involvement with lymphoplasmacytic cells. […] Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. […] Laboratory workup of suspected Waldenstrm Macroglobulinemia (WM) is enumerated in Table 1. […] Any mature B cell or plasma cell malignancy can potentially produce monoclonal IgM and hence, careful consideration of the differential diagnosis is vital. […] IgM MGUS is defined by presence of serum IgM concentration 3.0 g/dL, absence of lymphadenopathy, end organ damage and 10% marrow infiltration. It is important to do the confirmatory bone marrow biopsy to differentiate IgM MGUS from WM.
#46 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/view/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] The rationale for using a higher cutoff of LPL infiltration (10%) to define WM by the Mayo Clinic criteria stems from the high prevalence of IgM MGUS and low rate of its progression to active WM (1.5%/year). […] The presence of the MYD88 mutation favors the diagnosis of WM.
#47 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
Waldenstrms Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and 10% bone marrow involvement with lymphoplasmacytic cells. […] Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. […] Laboratory workup of suspected Waldenstrm Macroglobulinemia (WM) is enumerated in Table 1. […] Any mature B cell or plasma cell malignancy can potentially produce monoclonal IgM and hence, careful consideration of the differential diagnosis is vital. […] IgM MGUS is defined by presence of serum IgM concentration 3.0 g/dL, absence of lymphadenopathy, end organ damage and 10% marrow infiltration. It is important to do the confirmatory bone marrow biopsy to differentiate IgM MGUS from WM.
#48 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#49 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
At the Bing Center for Waldenstrm’s Macroglobulinemia at Dana-Farber Brigham Cancer Center, our specialists manage your diagnosis and treatment plan as a team. In our highly specialized center, your testing and care are coordinated from your first appointment. […] Because Waldenstrm’s macroglobulinemia (WM) is rare, it is sometimes misdiagnosed or mistreated. It can sometimes be mistaken for multiple myeloma or indolent lymphoma other B-cell malignancies. It’s important to be seen at or consult with a treatment center like ours that is experienced with this kind of cancer. […] The most accurate way to diagnose Waldenstrm’s is using a bone marrow biopsy supported by appropriate molecular testing for the MYD88 mutation. Our hematopathologists perform these tests every day and have deep experience in this area. The Bing Center lab discovered the role of the MYD88 mutation in WM and developed the molecular test for the most common MYD88 mutation (L265P).
#50 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
How We Diagnose Waldenstrom’s Macroglobulinemia […] At the Bing Center for WaldenstrÃ¶m’s Macroglobulinemia at Dana-Farber Brigham Cancer Center), our specialists manage your diagnosis and treatment plan as a team. Because we are a highly specialized center, your testing and care are coordinated from your first appointment. […] Because WaldenstrÃ¶m’s is uncommon, it is sometimes misdiagnosed. It’s important to be seen at â or consult with â a treatment center like ours that is dedicated to this kind of cancer. […] Some patients are completely asymptomatic when they are diagnosed. WM is often suspected after a blood test shows increased IgM in symptomatic patients. […] WaldenstrÃ¶m’s can be mistaken for multiple myeloma or indolent lymphoma, both of which are also B-cell malignancies. […] The most accurate way to diagnose WaldenstrÃ¶m’s is by a bone marrow biopsy and supported by appropriate molecular testing for the MYD88 mutation. At our center, our hematopathologists perform these studies on a daily basis and we have deep experience in this area. The MYD88 mutation in WM was discovered in the Bing Center Laboratories, and molecular testing for the most common MYD88 mutation (L265P) was developed in our laboratory. […] We also perform genomic testing for CXCR4 and TP53 which impact treatment decisions. The CXCR4 mutation in WM was discovered in the Bing Center and its importance in impacting WM disease presentation and treatment outcomes was first reported in studies from the Bing Center. […] Your doctor may also conduct imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins.
#51 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
BM biopsy is mandatory for a correct diagnosis to distinguish between the different forms of IgM monoclonal gammopathies, and it is particularly informative in distinguishing between WM and MYD88L265P MZL cases with IgM monoclonal protein. […] MYD88L265P assessment is mandatory during the diagnostic work-up of WM and related disorders, and must be done in BM samples, using ASqPCR or dPCR.
#52 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
The characteristic immunophenotypic features of WM clonal B-cells are intracytoplasmic and surface light-chain restriction, as well as surface expression of pan-B-cell antigens (CD19, CD20), together with CD22+dim, CD25+, CD27+ and IgM+. […] Although WM has a non-specific immunophenotype, CD5, CD23, CD10, cyclin D1, LEF1, and CD56 staining is helpful in excluding most differential diagnoses. […] Detection of MYD88 and, more recently, CXCR4 somatic mutations in ctDNA from PB of WM patients is an area under development, with initial promising results, showing a high concordance with tumor burden.
#53 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
The characteristic immunophenotypic features of WM clonal B-cells are intracytoplasmic and surface light-chain restriction, as well as surface expression of pan-B-cell antigens (CD19, CD20), together with CD22+dim, CD25+, CD27+ and IgM+. […] Although WM has a non-specific immunophenotype, CD5, CD23, CD10, cyclin D1, LEF1, and CD56 staining is helpful in excluding most differential diagnoses. […] Detection of MYD88 and, more recently, CXCR4 somatic mutations in ctDNA from PB of WM patients is an area under development, with initial promising results, showing a high concordance with tumor burden.
#54 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#55 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] Approximately 1 in 5 patients with WM have smoldering WM (SWM) at diagnosis, without the presence of symptoms or indications for initiating treatment per the consensus criteria from the Second International Workshop on WM. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population.
#56 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] Approximately 1 in 5 patients with WM have smoldering WM (SWM) at diagnosis, without the presence of symptoms or indications for initiating treatment per the consensus criteria from the Second International Workshop on WM. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population.
#57 Understanding the Treatment of WaldenstrÃ¶mâs Macroglobulinemia – The ASCO Post
https://ascopost.com/issues/september-25-2023/understanding-the-treatment-of-waldenstroem-s-macroglobulinemia/
Patients at one end of the disease spectrum do not require treatment: those with IgM monoclonal gammopathy of unknown significance (MGUS) and those with smoldering Waldenstrms macroglobulinemia. […] A diagnosis requires the presence of IgM monoclonal gammopathy, the presence of bone marrow infiltration by lymphocytes with plasmacytic differentiation in an intertrabecular pattern, and an immunophenotype supportive of both a lymphocytic component and a plasmacytic component. […] New patients should undergo a variety of laboratory tests and generally a bone marrow biopsy to identify mutations, which are informative for treatment selection. […] Since certain mutations can be prognostic and predictive, genotyping is important in the patient with Waldenstrms macroglobulinemia. […] Although observation is usually appropriate for patients with IgM MGUS and smoldering Waldenstrms macroglobulinemia, Dr. Nooka described scenarios that warrant the initiation of treatment:
#58 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] Approximately 1 in 5 patients with WM have smoldering WM (SWM) at diagnosis, without the presence of symptoms or indications for initiating treatment per the consensus criteria from the Second International Workshop on WM. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population.
#59 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] Approximately 1 in 5 patients with WM have smoldering WM (SWM) at diagnosis, without the presence of symptoms or indications for initiating treatment per the consensus criteria from the Second International Workshop on WM. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population.
#60 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
Waldenstrm macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. Per the Mayo Clinic criteria, WM is characterized by the presence of IgM monoclonal protein and 10% LPL infiltrate. […] Per the consensus criteria, evidence of IgM monoclonal gammopathy with histologic evidence of any degree of marrow involvement by LPL is sufficient to diagnose WM. […] Approximately 1 in 5 patients with WM have smoldering WM (SWM) at diagnosis, without the presence of symptoms or indications for initiating treatment per the consensus criteria from the Second International Workshop on WM. […] The distinction between SWM and active WM is important, because SWM does not require treatment and patients managed with active surveillance demonstrate comparable survival to the age- and sex-matched US population.
#61 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#62 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#63 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#64 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#65 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#66 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#67 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#68 Differential Diagnosis of Waldenström’s Macroglobulinemia | BLCTT
https://www.dovepress.com/differential-diagnosis-of-waldenstrms-macroglobulinemia-and-early-mana-peer-reviewed-fulltext-article-BLCTT
The rate of progression from IgM MGUS to WM is ~ 1.5% per year. […] If the clinical and pathological features do not offer a clear differentiation, a MYD88 mutation panel can be considered. […] The goal of early management in symptomatic WM is prompt recognition and treatment of complications of the disease. […] It is important to note that treatment is only indicated in patients symptomatic from adenopathy, organomegaly, neuropathy, hyperviscosity, cryoglobulinemia, cold agglutinin disease, cytopenias (Hb10 gm/dl, platelets 50 x 103/ L) or amyloidosis. […] The response to treatment should be monitored using the International Working Group on Waldenstroms Macroglobulinemia (IWWM) criteria during treatment. […] Plasmapheresis is utilized as a bridge to systemic therapy in patients with symptomatic hyperviscosity, symptomatic cryoglobulinemia or severe hemolysis resulting from cold agglutinin disease. […] Multiple effective therapies with comparable outcomes are now available for the treatment of WM. […] Patient preference, clinical presentation, co-morbidities, molecular features, and treatment goals should be considered before choosing appropriate initial therapy for newly diagnosed WM.
#69 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
We also perform genetic testing for CXCR4 and TP53 mutations, since these mutations impact treatment decisions. The Bing Center discovered the CXCR4 mutation in WM, as well as its importance in impacting WM disease presentation and treatment outcomes. […] Your doctor may also do imaging studies (CT scans, X-rays, and PET scans) of the chest, abdomen, and pelvis to look for an enlarged spleen or lymph nodes, or abnormal retinal veins. […] Because Waldenstrm’s involves the bone marrow, it is considered to be a whole body disease. This means that there is no staging process. Still, certain diagnostic factors, such as age, levels of IgM, hemoglobin, presence of MYD88, CXCR4, and TP53 mutations in WM cells, and platelets can help inform expected outcomes. […] Waldenstrm’s can be difficult to diagnose and properly treat. Reasons to consider a second opinion include: To confirm your diagnosis. If you have received a diagnosis and want to be treated at Dana-Farber Brigham Cancer Center. To determine the optimal therapy, and whether any is needed at this time. To learn more about your cancer from specialists who are world leaders in this disease, and who have treated hundreds of other patients like you. To learn if you’re eligible for a clinical trial.
#70 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Waldenstrom macroglobulinemia (WM) is a rare form of blood cancer that results in too many abnormal white blood cells, known as lymphoplasmacytic cells, in the bone marrow. […] Every year, around 1,0001,500 people in the United States receive a diagnosis of WM, according to the American Cancer Society (ACS). On average, people receive their WM diagnosis at the age of 70 years. […] Unlike other types of cancer, WM has no standard staging system. The extent of the disease is a factor when determining treatment or evaluating a persons outlook. […] Your outlook usually depends on the rate at which your disease is progressing. Doctors can use the International Prognostic Scoring System for Waldenstrom Macroglobulinemia (ISSWM) to help predict your outlook based on risk factors such as: age, blood hemoglobin level, platelet count, beta-2 microglobulin level, monoclonal IgM level.
#71 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
The International Prognostic Staging System for WM (IPSS-WM) is a validated model, often used in clinical practice, but needs to be reexamined in the current era. […] The prognosis of patients with WM is known to be impacted by certain clinical and laboratory features at initial presentation, with advanced age, elevated serum lactate dehydrogenase, and low serum albumin unfavorably affecting the outcome. […] The presence of the MYD88 mutation favors the diagnosis of WM.
#72 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Waldenstrom macroglobulinemia (WM) is a rare form of blood cancer that results in too many abnormal white blood cells, known as lymphoplasmacytic cells, in the bone marrow. […] Every year, around 1,0001,500 people in the United States receive a diagnosis of WM, according to the American Cancer Society (ACS). On average, people receive their WM diagnosis at the age of 70 years. […] Unlike other types of cancer, WM has no standard staging system. The extent of the disease is a factor when determining treatment or evaluating a persons outlook. […] Your outlook usually depends on the rate at which your disease is progressing. Doctors can use the International Prognostic Scoring System for Waldenstrom Macroglobulinemia (ISSWM) to help predict your outlook based on risk factors such as: age, blood hemoglobin level, platelet count, beta-2 microglobulin level, monoclonal IgM level.
#73 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Waldenstrom macroglobulinemia (WM) is a rare form of blood cancer that results in too many abnormal white blood cells, known as lymphoplasmacytic cells, in the bone marrow. […] Every year, around 1,0001,500 people in the United States receive a diagnosis of WM, according to the American Cancer Society (ACS). On average, people receive their WM diagnosis at the age of 70 years. […] Unlike other types of cancer, WM has no standard staging system. The extent of the disease is a factor when determining treatment or evaluating a persons outlook. […] Your outlook usually depends on the rate at which your disease is progressing. Doctors can use the International Prognostic Scoring System for Waldenstrom Macroglobulinemia (ISSWM) to help predict your outlook based on risk factors such as: age, blood hemoglobin level, platelet count, beta-2 microglobulin level, monoclonal IgM level.
#74 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Waldenstrom macroglobulinemia (WM) is a rare form of blood cancer that results in too many abnormal white blood cells, known as lymphoplasmacytic cells, in the bone marrow. […] Every year, around 1,0001,500 people in the United States receive a diagnosis of WM, according to the American Cancer Society (ACS). On average, people receive their WM diagnosis at the age of 70 years. […] Unlike other types of cancer, WM has no standard staging system. The extent of the disease is a factor when determining treatment or evaluating a persons outlook. […] Your outlook usually depends on the rate at which your disease is progressing. Doctors can use the International Prognostic Scoring System for Waldenstrom Macroglobulinemia (ISSWM) to help predict your outlook based on risk factors such as: age, blood hemoglobin level, platelet count, beta-2 microglobulin level, monoclonal IgM level.
#75 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Waldenstrom macroglobulinemia (WM) is a rare form of blood cancer that results in too many abnormal white blood cells, known as lymphoplasmacytic cells, in the bone marrow. […] Every year, around 1,0001,500 people in the United States receive a diagnosis of WM, according to the American Cancer Society (ACS). On average, people receive their WM diagnosis at the age of 70 years. […] Unlike other types of cancer, WM has no standard staging system. The extent of the disease is a factor when determining treatment or evaluating a persons outlook. […] Your outlook usually depends on the rate at which your disease is progressing. Doctors can use the International Prognostic Scoring System for Waldenstrom Macroglobulinemia (ISSWM) to help predict your outlook based on risk factors such as: age, blood hemoglobin level, platelet count, beta-2 microglobulin level, monoclonal IgM level.
#76 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Waldenstrom macroglobulinemia (WM) is a rare form of blood cancer that results in too many abnormal white blood cells, known as lymphoplasmacytic cells, in the bone marrow. […] Every year, around 1,0001,500 people in the United States receive a diagnosis of WM, according to the American Cancer Society (ACS). On average, people receive their WM diagnosis at the age of 70 years. […] Unlike other types of cancer, WM has no standard staging system. The extent of the disease is a factor when determining treatment or evaluating a persons outlook. […] Your outlook usually depends on the rate at which your disease is progressing. Doctors can use the International Prognostic Scoring System for Waldenstrom Macroglobulinemia (ISSWM) to help predict your outlook based on risk factors such as: age, blood hemoglobin level, platelet count, beta-2 microglobulin level, monoclonal IgM level.
#77 Waldenstromâs Macroglobulinemia Prognosis: Life Expectancy and Outlook
https://www.healthline.com/health/waldenstrom-macroglobulinemia/progression-and-outlook-for-waldenstrom-macroglobulinemia
Doctors score these factors to put people with WM into three risk groups: low, intermediate, and high. This helps doctors choose treatments and assess individual outlooks. […] According to the ACS, the 5-year survival rates are: 87% for low risk groups, 68% for intermediate risk groups, 36% for high risk groups. […] WM doesnt have a standard staging system like other cancers do. That means the amount of cancer in the body does not determine a persons outlook. Doctors look at other factors, including age, blood cell counts, and specific factors in the blood, to determine a persons outlook. […] The outlook for people with WM has improved in recent decades. According to the National Cancer Institute, the overall relative 5-year survival is about 78%.
#78 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
The International Prognostic Staging System for WM (IPSS-WM) is a validated model, often used in clinical practice, but needs to be reexamined in the current era. […] The prognosis of patients with WM is known to be impacted by certain clinical and laboratory features at initial presentation, with advanced age, elevated serum lactate dehydrogenase, and low serum albumin unfavorably affecting the outcome. […] The presence of the MYD88 mutation favors the diagnosis of WM.
#79 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
The International Prognostic Staging System for WM (IPSS-WM) is a validated model, often used in clinical practice, but needs to be reexamined in the current era. […] The prognosis of patients with WM is known to be impacted by certain clinical and laboratory features at initial presentation, with advanced age, elevated serum lactate dehydrogenase, and low serum albumin unfavorably affecting the outcome. […] The presence of the MYD88 mutation favors the diagnosis of WM.
#80 Diagnosis and Risk Stratification in WaldenstrÃ¶m Macroglobulinemia in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 7 (2024)
https://jnccn.org/abstract/journals/jnccn/22/7/article-e247024.xml
The International Prognostic Staging System for WM (IPSS-WM) is a validated model, often used in clinical practice, but needs to be reexamined in the current era. […] The prognosis of patients with WM is known to be impacted by certain clinical and laboratory features at initial presentation, with advanced age, elevated serum lactate dehydrogenase, and low serum albumin unfavorably affecting the outcome. […] The presence of the MYD88 mutation favors the diagnosis of WM.
#81 Bing Center for Waldenstrom’s Macroglobulinemia – How We Diagnose Waldenstrom’s Macroglobulinemia
http://waldenstroms.com/about-wm/how-we-diagnose-wm
In WM, targeted therapies are now focusing on specific molecular changes in WM cells. Since 2012, we have been actively investigating the importance of MYD88, CXCR4 and TP53 tumor mutations in people with WaldenstrÃ¶m’s and developed genomic specific guidelines for using genomic based studies to inform treatment decisions, Importantly, we also understand which tests are best able to identify MYD88, CXCR4 and TP53 mutations, and have performed extensive validation studies for MYD88 and CXCR4 mutation testing. […] Because WaldenstrÃ¶m’s involves the bone marrow, it is considered to be a disease of the whole body. This means that there is no staging process. Still, certain diagnostic factors, such as age, levels of IgM, hemoglobin, and platelets can be predictive in terms of outcomes, as can MYD88, CXCR4 and TP53 mutations in WM cells.
#82 Understanding the Treatment of WaldenstrÃ¶mâs Macroglobulinemia – The ASCO Post
https://ascopost.com/issues/september-25-2023/understanding-the-treatment-of-waldenstroem-s-macroglobulinemia/
If these findings can be tied to the underlying disease process, thats a trigger for me to start treatment. […] The activity of these regimens is not affected by mutation status, but they are associated with certain treatment-related side effects; therefore, treatment should be individualized. […] The mutation status helps us understand which patients should be treated with a BTK inhibitor, vs fixed-duration chemotherapy, where the outcome is not affected by mutation status, Dr. Nooka explained. […] Check MYD88 mutation status prior to starting ibrutinib. […] The BTK inhibitor zanubrutinib has advanced the indefinite-duration approach. […] There have been no randomized comparisons between the BTK inhibitorbased indefinite-duration regimens and the chemotherapy-based fixed-duration regimens, but an analysis of age-matched patient data has been conducted by an international team of collaborators.
#83 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
Therefore, a BM biopsy remains mandatory for the differential diagnosis between WM, IgM-MGUS and other B-cell lymphoproliferative disorders (B-LPDs). […] Multiparametric flow cytometry (MFC) and molecular techniques may help to confirm the diagnosis, especially to discriminate WM from other IgM-secreting disorders. […] MYD88L265P assessment is considered crucial to discriminate between WM and other B-LPDs with overlapping clinical features. […] Therefore, most groups currently agree that it may provide prognostic information about the risk of progression and the indication of the BM biopsy should be discussed. […] We aim to provide consensus recommendations of the European Consortium of Waldenstrms Macroglobulinemia (ECWM) on diagnostics in this lymphoma subtype. […] The work-up of suspected newly diagnosed WM patients should include pathological, MFC, and molecular studies guided by the recommendations shown in Table 4.
#84 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
However, the cut-off point of 1.5g/dl could be misleading since in WM, there is no concordance between BM infiltration, IgM levels and patient symptoms. […] Consequently, although the value of BM assessment in asymptomatic individuals is not fully established, most groups currently agree that it may provide prognostic information about the risk of progression and the indication of the BM biopsy should be discussed. […] We aim to provide consensus recommendations of the European Consortium of Waldenstrms Macroglobulinemia (ECWM) on diagnostics in this lymphoma subtype. […] We will discuss the basic and essential procedures that must be performed by local centers for the diagnosis and initial evaluation of WM patients, as well as more complex techniques that should be considered for precise pre-treatment evaluation, disease monitoring and research studies to be carried out in referral centers.
#85 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
Therefore, a BM biopsy remains mandatory for the differential diagnosis between WM, IgM-MGUS and other B-cell lymphoproliferative disorders (B-LPDs). […] Multiparametric flow cytometry (MFC) and molecular techniques may help to confirm the diagnosis, especially to discriminate WM from other IgM-secreting disorders. […] A progressive increase in the number of light-chain-isotype-positive B-cells from IgM-MGUS to smoldering and to symptomatic WM has been demonstrated. […] However, the pattern of antigen expression and the relative fractions of individual marker expressing clonal B-cells remain stable during disease progression. […] Asymptomatic patients with IgM monoclonal component below 1.5g/dl (or 15g/l) and normal serum free light-chain ratio have a very low-risk of progression to overt WM or other lymphoproliferative malignancies, and BM biopsy is not generally recommended at this stage, outside the context of clinical trials, and in the absence of any potential IgM-related symptom.
#86 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
However, the cut-off point of 1.5g/dl could be misleading since in WM, there is no concordance between BM infiltration, IgM levels and patient symptoms. […] Consequently, although the value of BM assessment in asymptomatic individuals is not fully established, most groups currently agree that it may provide prognostic information about the risk of progression and the indication of the BM biopsy should be discussed. […] We aim to provide consensus recommendations of the European Consortium of Waldenstrms Macroglobulinemia (ECWM) on diagnostics in this lymphoma subtype. […] We will discuss the basic and essential procedures that must be performed by local centers for the diagnosis and initial evaluation of WM patients, as well as more complex techniques that should be considered for precise pre-treatment evaluation, disease monitoring and research studies to be carried out in referral centers.
#87 Understanding the Treatment of WaldenstrÃ¶mâs Macroglobulinemia – The ASCO Post
https://ascopost.com/issues/september-25-2023/understanding-the-treatment-of-waldenstroem-s-macroglobulinemia/
If these findings can be tied to the underlying disease process, thats a trigger for me to start treatment. […] The activity of these regimens is not affected by mutation status, but they are associated with certain treatment-related side effects; therefore, treatment should be individualized. […] The mutation status helps us understand which patients should be treated with a BTK inhibitor, vs fixed-duration chemotherapy, where the outcome is not affected by mutation status, Dr. Nooka explained. […] Check MYD88 mutation status prior to starting ibrutinib. […] The BTK inhibitor zanubrutinib has advanced the indefinite-duration approach. […] There have been no randomized comparisons between the BTK inhibitorbased indefinite-duration regimens and the chemotherapy-based fixed-duration regimens, but an analysis of age-matched patient data has been conducted by an international team of collaborators.
#88 Understanding the Treatment of WaldenstrÃ¶mâs Macroglobulinemia – The ASCO Post
https://ascopost.com/issues/september-25-2023/understanding-the-treatment-of-waldenstroem-s-macroglobulinemia/
If these findings can be tied to the underlying disease process, thats a trigger for me to start treatment. […] The activity of these regimens is not affected by mutation status, but they are associated with certain treatment-related side effects; therefore, treatment should be individualized. […] The mutation status helps us understand which patients should be treated with a BTK inhibitor, vs fixed-duration chemotherapy, where the outcome is not affected by mutation status, Dr. Nooka explained. […] Check MYD88 mutation status prior to starting ibrutinib. […] The BTK inhibitor zanubrutinib has advanced the indefinite-duration approach. […] There have been no randomized comparisons between the BTK inhibitorbased indefinite-duration regimens and the chemotherapy-based fixed-duration regimens, but an analysis of age-matched patient data has been conducted by an international team of collaborators.
#89 Understanding the Treatment of WaldenstrÃ¶mâs Macroglobulinemia – The ASCO Post
https://ascopost.com/issues/september-25-2023/understanding-the-treatment-of-waldenstroem-s-macroglobulinemia/
If these findings can be tied to the underlying disease process, thats a trigger for me to start treatment. […] The activity of these regimens is not affected by mutation status, but they are associated with certain treatment-related side effects; therefore, treatment should be individualized. […] The mutation status helps us understand which patients should be treated with a BTK inhibitor, vs fixed-duration chemotherapy, where the outcome is not affected by mutation status, Dr. Nooka explained. […] Check MYD88 mutation status prior to starting ibrutinib. […] The BTK inhibitor zanubrutinib has advanced the indefinite-duration approach. […] There have been no randomized comparisons between the BTK inhibitorbased indefinite-duration regimens and the chemotherapy-based fixed-duration regimens, but an analysis of age-matched patient data has been conducted by an international team of collaborators.
#90 Diagnosis and Management of Waldenstromâs Macroglobulinemia – Curetalks
https://www.curetalks.com/waldenstroms-macroglobulinemia/
So, as you can see we are studying to have genomic profile into account when we make treatment decisions in these patients. […] So, I think its an exciting time for Waldenstroms and we have an exciting time for the last 10 years. […] So, I think this new generation of clinical trials combining BTK Inhibitors with a finite duration of the therapy is actually a very important study and one of an examples is an Canadian study that is actually looking at Acalabrutinib, Bendamustine and Rituximab and Acalabrutinib will be in for about a year and then everybody will stop therapy. […] So, the use of the isotopes is not new precisely but what is new I think is the fact that how they are taking advantage of the profile, of the membrane of the malignant cells to be able to deliver the real isotopes and stuff in a significant manner.
#91 Diagnosis and Management of Waldenstromâs Macroglobulinemia – Curetalks
https://www.curetalks.com/waldenstroms-macroglobulinemia/
So, when do we need to treat patients with Waldenstroms and the current approach is to treat patients who are symptomatic for one, we have to have symptoms that are severe enough to alter the patients quality of life. […] So, I think an important aspect of all this is to how well the patient responds to treatment in that sometimes its hard to predict, some patients will respond fantastically well and sometimes patients do not respond so well and again that is also something that we need to have in mind. […] I think the most important aspect in terms of prognosis is the age of the patient, patients who are younger tend to live longer and patients who are a bit older tend to shorter, that is life itself. […] So, these patients tend to have higher IGM levels, they tend to have higher risk of hyperviscosity and have even more bleeding complications as well.
#92 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia | Leukemia
https://www.nature.com/articles/s41375-022-01762-3
The diagnosis of Waldenstrms macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. […] This, together with the increasing variety of tools and techniques available, makes it necessary to have a practical guidance for clinicians to perform the initial evaluation of patients with WM. […] We provide the procedures for multiparametric flow cytometry, fluorescence in situ hybridization and molecular tests, and with this offer guidance for a standardized diagnostic work-up and methodological workflow of patients with IgM monoclonal gammopathy of uncertain significance, asymptomatic and symptomatic WM. […] The Consensus Panel Recommendations from the Second International Workshop on Waldenstrms Macroglobulinemia (WM) state that the diagnosis of WM requires the following clinical and pathological criteria: presence of infiltration of clonal lymphoplasmacytoid cells documented by bone marrow (BM) biopsy (lymphoplasmacytic lymphoma (LPL)) and presence of monoclonal IgM in the serum, irrespective of the M-protein size.
#93 Diagnostics in WaldenstrÃ¶mâs macroglobulinemia: a consensus statement of the European Consortium for WaldenstrÃ¶mâs Macroglobulinemia
https://pmc.ncbi.nlm.nih.gov/articles/PMC9898035/
The diagnosis of Waldenstrms macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. […] This, together with the increasing variety of tools and techniques available, makes it necessary to have a practical guidance for clinicians to perform the initial evaluation of patients with WM. […] In this paper, we present the consensus recommendations and laboratory requirements for the diagnosis of WM developed by the European Consortium of Waldenstrms Macroglobulinemia (ECWM), for both clinical practice as well as the research/academical setting. […] The Consensus Panel Recommendations from the Second International Workshop on Waldenstrms Macroglobulinemia (WM) state that the diagnosis of WM requires the following clinical and pathological criteria: presence of infiltration of clonal lymphoplasmacytoid cells documented by bone marrow (BM) biopsy (lymphoplasmacytic lymphoma (LPL)) and presence of monoclonal IgM in the serum, irrespective of the M-protein size.
#94 WaldenstrÃ¶m macroglobulinemia: 2021 update on diagnosis, risk stratification, and management – PubMed
https://pubmed.ncbi.nlm.nih.gov/33368476/
Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients and is found in the majority of IgM MGUS patients. […] Waldenstrm macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.
#95 Understanding the Treatment of WaldenstrÃ¶mâs Macroglobulinemia – The ASCO Post
https://ascopost.com/issues/september-25-2023/understanding-the-treatment-of-waldenstroem-s-macroglobulinemia/
Patients at one end of the disease spectrum do not require treatment: those with IgM monoclonal gammopathy of unknown significance (MGUS) and those with smoldering Waldenstrms macroglobulinemia. […] A diagnosis requires the presence of IgM monoclonal gammopathy, the presence of bone marrow infiltration by lymphocytes with plasmacytic differentiation in an intertrabecular pattern, and an immunophenotype supportive of both a lymphocytic component and a plasmacytic component. […] New patients should undergo a variety of laboratory tests and generally a bone marrow biopsy to identify mutations, which are informative for treatment selection. […] Since certain mutations can be prognostic and predictive, genotyping is important in the patient with Waldenstrms macroglobulinemia. […] Although observation is usually appropriate for patients with IgM MGUS and smoldering Waldenstrms macroglobulinemia, Dr. Nooka described scenarios that warrant the initiation of treatment:
#96 How We Diagnose WaldenstrÃ¶m’s Macroglobulinemia | Dana-Farber Cancer Institute
https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia/diagnosis
At the Bing Center for Waldenstrm’s Macroglobulinemia at Dana-Farber Brigham Cancer Center, our specialists manage your diagnosis and treatment plan as a team. In our highly specialized center, your testing and care are coordinated from your first appointment. […] Because Waldenstrm’s macroglobulinemia (WM) is rare, it is sometimes misdiagnosed or mistreated. It can sometimes be mistaken for multiple myeloma or indolent lymphoma other B-cell malignancies. It’s important to be seen at or consult with a treatment center like ours that is experienced with this kind of cancer. […] The most accurate way to diagnose Waldenstrm’s is using a bone marrow biopsy supported by appropriate molecular testing for the MYD88 mutation. Our hematopathologists perform these tests every day and have deep experience in this area. The Bing Center lab discovered the role of the MYD88 mutation in WM and developed the molecular test for the most common MYD88 mutation (L265P).