Materiały źródłowe

• #1 The Epidemiology of Waldenström Macroglobulinemia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10351685/

  Waldenstrm macroglobulinemia (WM) is a rare subtype of non-Hodgkin lymphoma characterized by the presence of lymphoplasmacytic lymphoma (LPL) in the bone marrow accompanied by a monoclonal immunoglobulin type M (IgM) in the serum. WM became reportable in the US as a malignancy in 1988. Very little systematic research was conducted prior to 2000 to characterize incidence, clinical characteristics, risk factors or diagnostic and prognostic criteria. Since the inaugural meeting of the International Workshop on Waldenstrms Macroglobulinemia (IWWM) in 2000, WM has become the focus of a steadily increasing and productive body of research. […] According to the current WHO classification, WM represents the most common subtype of lymphoplasmacytic lymphoma (LPL), where the remaining LPL cases are classified as non-WM type LPL. Together, WM/LPL accounts for approximately 2% of newly diagnosed NHL in the US. The overall age-adjusted incidence of WM in the US general population is 0.36 per 100,000. The complexity of WM as a clinicopathologically-defined entity is illustrated by differences in the proportion of WM and LPL reported by WHO pathologists and observed in SEER data.

• #2 Orphanet: Waldenström macroglobulinemia

  https://www.orpha.net/en/disease/detail/33226

  Waldenstrm macroglobulinemia (WM) has an overall incidence ranging between 1 in 123,000 and 1 in 263,000 persons/year in Europe and the USA, respectively and represents approximately 1-2% of all hematological malignancies. […] The median age at diagnosis is 72 years, and it is twice as frequent in men.

• #3 The epidemiology of Waldenström macroglobulinemia – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37099032/

  Waldenstrm macroglobulinemia (WM) is a rare subtype of non-Hodgkin lymphoma characterized by the presence of lymphoplasmacytic lymphoma (LPL) in the bone marrow accompanied by a monoclonal immunoglobulin type M (IgM) in the serum. WM was first described only 80 years ago and became reportable in the US as a malignancy in 1988. Very little systematic research was conducted prior to 2000 to characterize incidence, clinical characteristics, risk factors or diagnostic and prognostic criteria, and there were essentially no WM-specific clinical interventional trials. Since the inaugural meeting of the International Workshop in Waldenstrm’s Macroglobulinemia (IWWM) in 2000, WM has become the focus of a steadily increasing and productive body of research, engaging a growing number of investigators throughout the world.

• #4 Key Statistics About Waldenstrom Macroglobulinemia | American Cancer Society

  https://www.cancer.org/cancer/types/waldenstrom-macroglobulinemia/about/key-statistics.html

  Waldenstrom macroglobulinemia (WM) is rare, with an incidence rate of about 3 cases per million people per year in the United States. About 1,000 to 1,500 people are diagnosed with WM each year in the United States. […] WM is more common in men than it is in women, and it is much more common among White people than Black people. […] There are few cases of WM in younger people, but the chance of developing this disease goes up as people get older. The average age of people when they are diagnosed with WM is 70.

• #5 Pathophysiology and Treatments of Complications of Waldenström’s Macroglobulinemia | Published in Clinical Hematology International

  https://chi.scholasticahq.com/article/124268-pathophysiology-and-treatments-of-complications-of-waldenstrom-s-macroglobulinemia

  Waldenstroms macroglobulinemia (WM) is a rare hematologic malignancy that consists of lymphoplasmacytic cells (LPCs) in the bone marrow and a monoclonal immunoglobulin (Ig) M spike in the serum. The prevalence of WM is only 1,000-1,500 new cases per year in the United States and accounts for only 2% of all hematologic malignancies. […] Despite the rarity of the disease, there are a variety of unique complications usually present at the time of diagnosis. The pathophysiology of these complications is mostly related to the pentameric structure of the IgM being produced by LPCs, and include amyloidosis, autoimmune hemolytic anemia, renal disease, increased risk of bleeding, neuropathy and organomegaly. […] Understanding the pathophysiology of these complications is important in indicating treatments for them along with assessing the response.

• #6 Epidemiology of Waldenström Macroglobulinemia | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-319-22584-5_9

  Waldenstrms Macroglobulinemia (WM) is a rare malignancy, accounting for only 12 % of all hematologic tumors, with an incidence of 34 per million people per year. […] Due to the rarity of WM, acquiring epidemiological knowledge of the disease has been a challenge. […] WM is a disease of the elderly, with the median age of diagnosis is around 70 years. […] Known risk factors for WM include family history and chronic antigen stimulation (i.e. chronic infections and autoimmune diseases), and IgM MGUS, which can be thought of as an early state of WM. […] Over recent decades there have been evidence of increased survival over time. […] The incidence and survival of Waldenstrms Macroglobulinaemia in South East England. […] Survival trends in Waldenstrm macroglobulinemia: an analysis of the Surveillance, Epidemiology and End Results database.

• #7 Waldenström’s macroglobulinemia epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Waldenstr%C3%B6m%27s_macroglobulinemia_epidemiology_and_demographics

  Waldenstrom’s macroglobulinemia is one of the rare subtypes of NHL accounting just 1-2% of it. […] The prevalence of Waldenstrom’s macroglobulinemia is estimated to be 1000-1,500 cases in United States annually. […] WM accounts for approximately 1% to 2% of hematologic cancers in United States and Western Europe. […] Worldwide, the overall age-adjusted incidence of Waldenstrom’s macroglobulinemia is 0.38 cases per 100,000 persons annually, increasing with age to 2.85 in patients above 80 years. […] Incidence of WM is approximately 8.3 cases per million persons per year. […] WM incidence is approximately 10-fold lower in Asia. […] Majority of WM patients are Caucasians, with other ethnic groups accounting for only 5 percent of cases. […] The age-adjusted incidence rate for males is 0.92 per 100,000 person-years.

• #8 Waldenstrom Macroglobulinemia: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/207097-overview

  Waldenstrm macroglobulinemia is a relatively rare condition, with the 1500 cases diagnosed per year in the United States accounting for approximately 2% of hematologic malignancies. The incidence rate for Waldenstrm macroglobulinemia in the United States is higher among whites, with people of African descent representing only 5% of all patients. In the United Kingdom, the annual incidence of the disease is 10.3 per million. […] Waldenstrm macroglobulinemia is a disease of the elderly. Most patients present in the seventh or eighth decade of life. The median age at diagnosis in the United States is 65 years, with a slight male predominance.

• #9 The Epidemiology of Waldenström Macroglobulinemia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10351685/

  Monoclonal gammopathy of undetermined significance of IgM type (IgM MGUS) is a well-established risk factor for WM. IgM MGUS has been shown to progress to WM or a related B-cell malignancy at a rate of about 1.5% per year. […] Accumulating evidence strongly supports a role for chronic immune stimulation in the etiology of WM. A nested case-control study in a cohort of over 700,000 US veterans demonstrated an increased risk for WM among veterans infected with HCV. […] The incidence rates reported for WM and LPL combined in northern Sweden from 2000 – 2012 were 50% -75% higher compared to Sweden overall, which is two- to three-fold higher than the combined rate in the US for the same period. […] During the eighty years following the first description of WM, substantial progress has been made in understanding the epidemiology and etiology of WM, as well as advances in methods for diagnosis, prognostic assessment and therapy. The incidence, distribution and survival patterns of WM/LPL show distinctive variation by age, sex, and race/ethnicity. These patterns strongly suggest that genetic, infectious, environmental, and possibly lifestyle factors in addition to other host factors are important in the etiology of WM/LPL.

• #10 Incidence, prevalence, mortality, and causes of death in Waldenström macroglobulinemia: a nationwide, population-based cohort study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07120-9

  The epidemiological features of Waldenstrm macroglobulinemia (WM) have seldom been investigated at a national level, particularly in East Asia. The goal of our study is to present the incidence, prevalence, mortality, survival with competing risks, and causes of death of patients with WM. […] The total number of patients newly diagnosed with WM during the study period was 427, with a male-to-female ratio of 3.2:1. The incidence increased from 0.03 to 0.10 per 105 between 2003 and 2016, and the prevalence was 0.42 per 105 in 2016. A total of 217 patients with WM died during the study period (standardized mortality ratio=7.57), and the overall survival (OS) of WM patients was 47.5%. […] The national incidence of WM in Korea, a racially homogeneous country in Asia, was lower than that in previous reports from other countries, reflecting ethnic disparities. However, the incidence increased, and mortality was the highest ever reported. The main cause of death was WM in itself. This study reflects the need for greater awareness of WM, particularly in Asian countries.

• #11 The Epidemiology of Waldenström Macroglobulinemia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10351685/

  While rare overall, WM/LPL incidence increases markedly with age, sex and race and ethnicity. WM is strongly age-dependent and is almost never diagnosed under age 30. The overall incidence of WM is approximately two-fold higher in males compared to females in the US. […] The incidence of WM has increased substantially in the US over the three decades since WM data became reportable. The annual age-adjusted incidence increased by 65% between 1990 and 2019. The increase is more pronounced in males relative to females and in the elderly. […] Familial clustering in a variety of pedigree configurations has been observed for 60 years. Within these kindred, relatives of WM patients presented with WM, related B-cell malignancies and/or IgM MGUS. WM patients in a large clinical case series reported a high prevalence of either WM or another B-cell disorder in first-degree relatives.

• #12 Incidence, prevalence, mortality, and causes of death in Waldenström macroglobulinemia: a nationwide, population-based cohort study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07120-9

  According to population-based studies, the survival trends in patients with WM have improved within the last decade. However, variations in geographical location and ethnicity affect the survival of WM patients. […] The present study was based on a comprehensive database, which is run by the National Health Insurance (NHI) of the Korean government. This database contains all the records of healthcare utilization among inpatients and outpatients. Therefore, this database was considered appropriate to reliably investigate the epidemiological features of WM. […] The overall incidence and the age- and gender-specific annual incidence of WM are presented in Table 1 and Fig. 1a. The total number of patients with WM in Korea between 2003 and 2016 was 427, comprising 326 men and 101 women. The annual incidence increased from 0.03 to 0.10 per 100,000 people from 2003 through 2016.

• #13 Diagnosis and Risk Stratification in Waldenström 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 an IgM-secreting lymphoplasmacytic lymphoma (LPL), with up to 1,500 new cases diagnosed annually in the United States. The median age at diagnosis is approximately 70 years, although a steady increase in incidence is notable with advancing age. A consistently higher prevalence in men and Caucasians compared with other races is also striking. […] 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. […] Approximately 1 in 5 patients with WM have a first-degree relative with a B-cell disorder. […] The diagnosis of IgM MGUS versus WM hinges on the degree of bone marrow infiltration by LPL. […] 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.

• #14 Managing Waldenström’s macroglobulinemia with BTK inhibitors | Leukemia

  https://www.nature.com/articles/s41375-022-01732-9

  Waldenstrms macroglobulinemia (WM) is a rare indolent B-cell lymphoma characterized primarily by bone marrow infiltration by lymphoplasmacytic cells and IgM monoclonal gammopathy. It comprises approximately 12% of all cases of non-Hodgkins lymphoma in Europe and the USA, with an incidence of ~4 cases per 1 million person-years. Prevalence is higher in males than in females and in Caucasians versus other races, and increases with age, with most WM patients aged 65 years at the time of diagnosis. It is incurable, with a median survival of 10-12 years, and many patients die from causes unrelated to the disease. […] The emergence of more effective treatments over the last two decades has resulted in improved outcomes for patients. […] Several ongoing trials in WM are investigating the potential for BTKi use in combination with established and novel targeted agents.

• #15 SciELO Brazil – Waldenström’s macroglobulinemia – a review Waldenström’s macroglobulinemia – a review

  https://www.scielo.br/j/ramb/a/sFTFrpnLqRXq8RfHvD7BpMk/

  Waldenstrm’s macroglobulinemia (WM) is a rare disease with a typically indolent clinical course, affecting mainly individuals aged between 63 and 68 years. […] WM has an estimated incidence of 3 cases/million/year, accounting for about 2% of all hematological cancers. […] There is a higher incidence in individuals aged between 63 and 68 years. […] Approximately 60% of patients are men, and it is more common in caucasian individuals. […] The average survival is 5 years, however, approximately 10% of patients survive up to 15 years. […] As the disease is mainly diagnosed in old age, about 50% of patients die due to comorbidities not related directly to WM.

• #16 Waldenström’s macroglobulinemia epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Waldenstr%C3%B6m%27s_macroglobulinemia_epidemiology_and_demographics

  The age-adjusted incidence rate for females is 0.30 per 100,000 person-years. […] Combined age and sex-adjusted incidence is 0.57 per 100,000 person-years. […] The incidence of Waldenstrom’s macroglobulinemia increases after 50 years of age. […] The median age at diagnosis is 65 years. […] Men are twice more likely than women to develop WM (5.4 vs. 2.7 per million, respectively). […] Higher incidence in whites (4.1 per million per year) comparative to blacks (1.8 per million per year) and in past 20 years, incidence in whites has elevated.

• #17 Waldenström’s Macroglobulinemia | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/types/waldenstroms-macroglobulinemia

  Waldenstrm’s is uncommon. About 2,000 to 3,000 people are diagnosed each year in the United States. […] The chance of developing Waldenstrm’s increases with age; the mid-60’s is the average age at which most patients are diagnosed. […] Up to 20% of WM patients have an Ashkenazi Jewish heritage. […] Up to 20% of WM patients have a first- or second-degree relative with WM or another lymphoma, multiple myeloma, or chronic lymphocytic leukemia. This suggests there may be a genetic risk factor. We are leading research studies in collaboration with the National Institutes of Health (NIH) to identify the role genetics plays in WM. […] Our colleagues in the Center for Early Detection and Interception of Blood Cancers (formerly the Center for Prevention of Progression), led by Irene Ghobrial, MD, are studying the genetic and epigenetic factors that characterize conditions that are often precancerous to WM, such as smoldering Waldenstrm’s macroglobulinemia and Monoclonal Gammopathy of Undetermined Significance (MGUS). The Center’s mission is to understand, at the molecular level, why some patients go on to develop WM disease, while others do not and to develop targeted therapies to prevent progression, or even eliminate the disease before it leads to symptoms.

• #18 Management of Waldenstrom’s macroglobulinaemia – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/management-of-waldenstroms-macroglobulinaemia

  Waldenstrom’s macroglobulinaemia (WM) is a rare type of non-hodgkin lymphoma, characterised by the presence of a monoclonal IgM protein. Approximately 350 people are diagnosed with WM in the UK each year. It is more common in men than it is in women, and it is much more common among people of white ethnicity than people of black ethnicity. In approximately 20% of cases, there is a familial predisposition. Approximately 1.5–2.0% of healthy individuals with an IgM-MGUS (monoclonal gammopathy of undetermined significance) progress to WM each year. As with other chronic B-cell malignancies, WM tends to affect older people, with an average age at diagnosis of 70 years. It is incurable, with median survival of 10–12 years; however, many patients die from causes unrelated to the disease. […] Symptoms of disease can arise from the physico-chemical and immunological effects of the monoclonal IgM protein (paraprotein) or from infiltration of the bone marrow and lymphoid organs; in the absence of symptoms, patients can be monitored with active surveillance.

• #19 Waldenstrom macroglobulinemia

  https://atlasgeneticsoncology.org/haematological/2043/waldenstrom-macroglobulinemia

  Epidemiology: Incidence: 3.4 per million in males, 1.7 per million in females (Hunter Z et al, 2017) Overall survival: 78% (5y) (Wang H et al, 2012) Median age at diagnosis: 65y (Wang H et al, 2012) Ethnicity: More common in Caucasians (90% of cases) than other ethnic groups; uncommon in Blacks (5% of cases) (Wang H et al, 2012). Those of Ashkenazi descent may be predisposed (Hanzis C et al, 2011) […] Advances in diagnostics and therapeutics have resulted in improved survival rates (Treon SP et al, 2014). Between the 1980s and the 21st century, 5- and 10-year survival rates have improved from 67% to 78% and 49% to 66%, respectively (Castillo JJ et al, 2014).

• #20 Homo sapiens molecular nosology – Waldenstrom macroglobulinemia

  https://www.ufrgs.br/imunovet/molecular_immunology/pathohomotissueblood_WM.html

  Epidemiology : 2% of all hematologic malignancies. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. WM is a disease of the elderly, with a median age of 63 years (range 25-92), with a slight predominance of males over females. […] The morbidity associated with WM is caused by the concurrence of 2 main components: tissue infiltration by neoplastic cells and, more importantly, the physicochemical and immunological properties of the monoclonal IgM. […] Prognosis : WM presents with a chronic, indolent course and a highly variable prognosis. The median survival reported in large series ranges from 5 to 7 years, although an observed survival of 9 years and a 10-year projected overall survival of 55% have been reported. Because WM is a rare disease, relatively few studies on prognosis have been conducted on large patient populations. Advanced age, anemia, and thrombocytopenia were correlated by univariate analysis with a poorer outcome in virtually all studies. Neutropenia and male sex, weight loss and cryoglobulinemia, albumin level and blood cell counts, serum 2-microglobulin level and IgM level 40 g/L, and hyperviscosity and 2-microglobulin level were also significantly correlated with survival.

• #21 Incidence, prevalence, mortality, and causes of death in Waldenström macroglobulinemia: a nationwide, population-based cohort study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07120-9

  The crude prevalence was 0.42 per 100,000 individuals, when calculated with the 2016 population as the denominator. The prevalence for men and women in 2016 were 0.62 and 0.21, respectively; the male-to-female ratio was 2.9:1, which was similar to that of incidence. […] The SMR for all WM patients was 7.57 (95% CI, 6.56 to 8.57), indicating that mortality in WM patients was significantly higher compared to that of the general population. […] The main cause of death in our cohort was directly attributable to WM, and the magnitude (48.57%) was almost two times higher than that of a previous report from the SEER database (25%).

• #22 Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment

  https://www.mdpi.com/2673-6357/3/4/44

  Transformation to an aggressive B-cell lymphoma is estimated to occur in 1–4% of patients with WM. The 5-, 10- and 15-year cumulative incidence rates of transformation were 1, 2 and 4% and 2, 5 and 6%, respectively, in the Dana-Farber Cancer Institute and the Mayo Clinic cohorts. An increased incidence of HT among patients with WM treated with nucleoside analogs was suggested in retrospective studies, but this was not confirmed in the WM1 trial. In the randomized WM1 trial that compared oral fludarabine and chlorambucil monotherapies as the frontline treatments, the 6-year cumulative incidence of HT was 8% in the fludarabine arm and 11% in the chlorambucil arm. In the Mayo Clinic cohort, prior nucleoside-analog-based therapy or rituximab-based therapies did not impact the risk of transformation. The rates of HT in patients treated with Bruton’s tyrosine kinase (BTK inhibitors) have not been reported to date.

• #23 Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment

  https://www.mdpi.com/2673-6357/3/4/44

  The data regarding the risk factors for the development of DLBCL in WM are scant. In recent years, two studies have demonstrated that the MYD88WT genotype was independently associated with a higher risk of HT. HT occurred in 15% and 1% of MYD88WT- and MYD88-mutated patients, respectively, in the study by Treon and colleagues, with 10-year cumulative incidence rates of 20% and 1%, respectively. In the study by Zanwar and colleagues, MYD88WT was the only factor associated with an increased risk of HT in a multivariate analysis (odds ratio (OR) 7, p = 0.003). The MYD88WT genotype was also associated with a shorter time to transformation (hazard ratio (HR) 7.9, p = 0.001).

• #24 Incidence and Risk of Secondary Malignancy in Patients with Waldenström Macroglobulinemia: A Population-Based Analysis | Published in Clinical Hematology International

  https://chi.scholasticahq.com/article/90436-incidence-and-risk-of-secondary-malignancy-in-patients-with-waldenstrom-macroglobulinemia-a-population-based-analysis

  An analysis of the Surveillance, Epidemiology, and End Results (SEER) database in 2015 demonstrated that patients with WM have a 49% increased risk of SMs compared to the general population. […] The overall risk of developing SM was significantly higher in WM patients compared to the general population (SIR= 1.53, 95% Cl=1.42-1.65, p 0.05) with an AER of 102.69 per 10,000 population. […] The risk of developing second primary solid tumors was higher than the general population (SIR= 1.12, 95% Cl= 1.02- 1.23, p 0.05) with an AER of 19.79. The overall risk of developing secondary hematological malignancies was also significantly increased (SIR= 4.95, 95% Cl 4.31-5.66, p 0.05) with an AER of 71.95. […] The risk for hematological malignancies was also increased (SIR 4.95): extra-nodal non-Hodgkin lymphoma (NHL) (SIR 11.3), NHL (SIR 8.29), acute lymphocytic leukemia (ALL) (SIR 8.04), lymphoma (SIR 7.98), nodal NHL (SIR 6.62), acute myeloid leukemia (AML) (SIR 4.22), acute non-lymphocytic leukemia (ANLL) (SIR 4.2), myeloid and monocytic leukemia (SIR 3.28), leukemia (SIR 2.01), and myeloma (SIR 1.93).

• #25 Waldenstrom macroglobulinemia: prognosis and management | Blood Cancer Journal

  https://www.nature.com/articles/bcj201528

  Several staging systems have been proposed to risk stratify patients with WM and to aid in prognosis. Given the wide selection of new treatments available for WM, a standardized scoring system has been developed recently, known as the International prognostic scoring system for Waldenstrom macroglobulinemia (IPSSWM), to help guide treatment based on prognosis in symptomatic patients.

• #26 Waldenström macroglobulinemia | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/waldenstrom-macroglobulinaemia?embed_domain=hackmd.io%252525252f%2525252540yipuafecsl2jsu8smr5njq%252525252fbnjhjgjghjghjgh&lang=us

  Waldenstrm macroglobulinemia is a rare condition, accounting for only 1% of all lymphoproliferative disorders. The incidence is 1500 cases per year in the United States. The median age of onset is 60-65 years. A minority of cases occur in late adolescence. […] The International Prognostic Scoring System for Waldenstrm macroglobulinemia is a predictive model for characterization of long-term outcome. Factors predicting survival are: age 65 years, hemoglobin 11.5 g/dL, platelet count 100 x 109/L, B2-microglobulin 3 mg/L, serum monoclonal protein concentration 70 g/L. […] Five-year survival rates for these categories are 87%, 68%, and 36% respectively.

• #27 Diagnosis and Risk Stratification in Waldenström 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 IPSS-WM derivation did not take the alternate causes of death into account and requires validation with a competing-risk analysis. […] The presence of CXCR4 mutations clearly leads to slower response as well as lower response rates with BTK inhibitors, but its differential impact on OS is yet to be observed in a large study in the Western population.

• #28 Management of Waldenstrom’s macroglobulinaemia – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/management-of-waldenstroms-macroglobulinaemia

  Patients with asymptomatic disease should be managed with an active surveillance strategy, which includes monitoring of clinical and laboratory findings every three to six months and appropriate immunisations. The median time for patients to progress to symptomatic disease is approximately five years and the main risk factors for progression are IgM paraprotein concentration, degree of bone marrow infiltration with lymphoplasmacytic cells and haemoglobin value.

• #29 Waldenstrom macroglobulinemia: What to expect during watch & wait? | Mayo Clinic Connect

  https://connect.mayoclinic.org/discussion/waldenstrom/?pg=8

  I prefer to refer to „watch and wait” as the „active surveillance” treatment option. Watch and wait feels like inaction and just waiting for the worst. Active surveillance is pro-active. Your condition may not require treatments like chemo, but you are still being actively monitored for any changes that may indicate a different treatment approach. […] When deciding on treatment options, your doctor will take many things into consideration like how slow or fast the condition progresses, your age, health status, personal preferences and treatment side effects. […] Just watch & wait for now. Next blood tests in October.

• #30 Simple headache revealed a rare lymphoma: Waldenstrom macroglobulinemia with unique markers: a case report and review of the literature | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-022-00107-6

  Waldenstrom macroglobulinemia (WM) is a rare lymphoma with an incidence rate of 3 per million people per year, with approximately 1000 to 1500 new cases diagnosed each year in the USA. […] WM is characterized by immunoglobulin M (IgM) monoclonal gammopathy, lymphocytic infiltration of bone marrow, and normocytic anemia due to bone marrow replacement. […] WM has lower median survival in older age groups, approximately 7 and 4 years for those 70-79 and those 80 or older, respectively. […] WM diagnosis depends on both non-invasive and invasive procedures. Non-invasive measures mainly consist of serum and 24-h urine collection for protein electrophoresis, Serum immunofixation to confirm the IgM heavy and light chains, and quantitative test for immunoglobulin G immunoglobulin A and IgM. […] Low volume lymphoma or hematological malignancies medical center along with inaccuracy estimate of WM incidence and prevalence are the main reasons behind the many undiagnosed WM cases despite the vast amount of cancer registry data provided.

• #31 Simple headache revealed a rare lymphoma: Waldenstrom macroglobulinemia with unique markers: a case report and review of the literature | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-022-00107-6

  The absence of the differentiation among IgM monoclonal gammopathy of asymptomatic WM and symptomatic WM requiring therapy treatment is the most critical limitation. […] With the continuous change in the diagnostic criteria, the treatment guidelines are in constant evolution. […] Yet, the main lines of treatment contain two main core treatments: treating the associated symptoms, especially hyperviscosity syndrome, and reducing tumor mass. […] Although affecting a decreasing portion of patients nowadays, hyperviscosity syndrome is rarely symptomatic, it makes treatment challenging. […] When hyperviscosity exists, plasma exchange should be carried out as a mainstay measure until systemic therapy successfully reduces the tumor mass and decreases the IgM protein serum concentration. […] We recommend physicians to have high yield dependence on BM biopsy and CT scans when WM is suspected.

• #32 Pathophysiology and Treatments of Complications of Waldenström’s Macroglobulinemia | Published in Clinical Hematology International

  https://chi.scholasticahq.com/article/124268-pathophysiology-and-treatments-of-complications-of-waldenstrom-s-macroglobulinemia

  The treatment goal of hyperviscosity syndrome includes rapidly reducing IgM levels to reduce serum viscosity, increase blood flow, and subsequently reduce symptoms and organ damage. […] Treatment of neurological complications of WM vary depending on the specific complication and its mechanism. […] Treatment for cryoglobulinemia in WM should be given to patients who are symptomatic or with end organ dysfunction. The goal in the acute setting in severely symptomatic patients is to eliminate immune complexes in the blood and start cytoreductive therapy. […] Given the rarity of WM, conducting randomized controlled trials to treat its complications can be difficult due to patient recruitment. As a result, there is no standard therapy for WM.

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