Białaczka włochatokomórkowa
Etiologia i przyczyny

Białaczka włochatokomórkowa (HCL) to rzadki, przewlekły nowotwór limfocytów B, stanowiący około 2% wszystkich białaczek, z roczną zachorowalnością około 1000 przypadków w USA. Kluczowym czynnikiem patogenetycznym jest mutacja somatyczna BRAF V600E, obecna u 80-90% pacjentów z klasyczną postacią HCL, prowadząca do konstytutywnej aktywacji szlaku RAF-MEK-ERK i zwiększonej przeżywalności komórek białaczkowych. Mutacja ta pozostaje stabilna przez cały przebieg choroby, co umożliwia zastosowanie inhibitorów BRAF w terapii. Dodatkowo obserwuje się utratę fragmentu chromosomu 7q, mutacje w genie IGHV (około 90% pacjentów) oraz nadekspresję cykliny D1. Wariant HCL (HCL-V) różni się genetycznie, nie posiada mutacji BRAF V600E, lecz mutacje w MAP2K1, co wiąże się z gorszą odpowiedzią na leczenie. Etiologia HCL jest wieloczynnikowa, obejmująca ekspozycję na pestycydy, herbicydy, rozpuszczalniki organiczne, promieniowanie jonizujące oraz czynniki infekcyjne, przy czym palenie tytoniu może mieć efekt ochronny.

Etiologia białaczki włochatokomórkowej

Białaczka włochatokomórkowa (Hairy Cell Leukemia, HCL) jest rzadkim, przewlekłym nowotworem układu krwiotwórczego, który wywodzi się z komórek B układu limfatycznego. Stanowi około 2% wszystkich białaczek, z około 1000 nowych przypadków diagnozowanych rocznie w Stanach Zjednoczonych12. Mimo postępu badań nad tą chorobą, dokładna etiologia HCL pozostaje nie w pełni poznana, choć zidentyfikowano szereg czynników genetycznych i środowiskowych, które mogą mieć związek z jej rozwojem34.

Podłoże genetyczne

Najważniejszym odkryciem dotyczącym patogenezy HCL było zidentyfikowanie mutacji BRAF V600E, obecnej u około 80-90% pacjentów z klasyczną postacią choroby56. Ta somatyczna mutacja punktowa w genie BRAF (proto-onkogen kodujący kinazę) prowadzi do konstytutywnej aktywacji szlaku sygnałowego RAF-MEK-ERK, co skutkuje zwiększoną przeżywalnością komórek i charakterystycznym fenotypem komórek białaczkowych54. Badanie z 2011 roku zidentyfikowało tę mutację u wszystkich 47 badanych pacjentów z HCL, podczas gdy nie wykryto jej u 193 pacjentów z innymi nowotworami z limfocytów B7.

Mutacja BRAF V600E pozostaje stabilna przez cały przebieg choroby, od początkowego rozpoznania do nawrotów, które mogą wystąpić nawet wiele lat po początkowej prezentacji5. Jest to kluczowe odkrycie, które zmieniło podejście do terapii HCL, umożliwiając zastosowanie inhibitorów BRAF jako potencjalnej opcji leczenia8.

Dodatkowo u pacjentów z HCL mogą występować inne zmiany genetyczne:

Warto zauważyć, że choć zidentyfikowano wariant HCL (HCL-V), jest on odrębną jednostką chorobową i nie zawiera mutacji BRAF V600E, ale raczej wykazuje mutacje w genie MAP2K1 (mitogen-activated protein kinase kinase 1)1112. Ta różnica genetyczna ma istotne znaczenie kliniczne, ponieważ wariant HCL gorzej odpowiada na standardowe leczenie13.

Komórkowe pochodzenie HCL

Białaczka włochatokomórkowa prawdopodobnie wywodzi się z późnej, aktywowanej komórki B pamięci, wykazującej cechy przedplazmatyczne lub pogerminalnego centrum41. Fenotypowo i morfologicznie komórki HCL nie przypominają żadnego normalnego stadium dojrzewania i rozwoju komórek B12.

Ze względu na morfologiczne i funkcjonalne podobieństwa między komórkami włochatymi a monocytami/makrofagami, początkowo uważano, że HCL wywodzi się z komórek siateczkowo-śródbłonkowych. Odkrycie rearanżacji genów immunoglobulinowych receptorów komórek B u pacjentów z tą chorobą wykazało, że HCL ma pochodzenie z komórek B12. Badania z użyciem przeciwciał monoklonalnych potwierdziły, że komórki włochate są dojrzałymi komórkami B12.

Czynniki ryzyka środowiskowe

Chociaż dokładna przyczyna HCL pozostaje nieznana, badania sugerują związek z ekspozycją na różne czynniki środowiskowe143:

  • Ekspozycja na pestycydy i herbicydy – badania epidemiologiczne wskazują na zwiększone ryzyko u osób narażonych na działanie pestycydów, zwłaszcza związków fosforoorganicznych315
  • Praca w rolnictwie – istnieje korelacja między pracą na farmie i w ogrodnictwie a zwiększonym ryzykiem rozwoju HCL75
  • Ekspozycja na Agent Orange – badacze sądzą, że istnieje związek między tym herbicydem a przewlekłymi białaczkami z komórek B i chłoniakami, w tym HCL616
  • Kontakt z rozpuszczalnikami organicznymi – szczególnie benzenem i innymi aromatycznymi rozpuszczalnikami1017
  • Ekspozycja na promieniowanie jonizujące – wyniki badań nie są jednoznaczne, ale sugerują możliwy związek1819
  • Kontakt z drewnem i pyłem drzewnym – niektóre badania wykazały związek między pracą z drewnem a zwiększonym ryzykiem HCL1020
  • Przebyta mononukleoza zakaźna – wymieniana jako możliwy czynnik etiologiczny10

Warto jednak zaznaczyć, że ekspozycja na dym tytoniowy, alkohol i otyłość nie wydają się być czynnikami ryzyka4. Co ciekawe, palenie może mieć odwrotną zależność, zwłaszcza u mężczyzn, choć dokładny mechanizm ochronny nie jest znany21.

Wirus HTLV-2

Ludzki wirus limfotropowy T typu 2 (HTLV-2) wyizolowano u niewielkiej liczby pacjentów z wariantem HCL. W latach 80. wirus HTLV-2 zidentyfikowano u pacjenta z chorobą limfoproliferacyjną komórek T; pacjent ten później rozwinął HCL, ale HTLV-2 nie został znaleziony w klonach komórek włochatych15. Nie ma jednak dowodów na to, że HTLV-2 powoduje jakikolwiek rodzaj nowotworu hematologicznego, w tym HCL15.

Czynniki demograficzne i rodzinne

Białaczka włochatokomórkowa ma charakterystyczny profil demograficzny622:

  • Wiek – HCL najczęściej dotyka osoby w wieku 40-70 lat, ze średnim wiekiem w momencie diagnozy wynoszącym około 55-58 lat622
  • Płeć – choroba występuje 4 razy częściej u mężczyzn niż u kobiet2322
  • Pochodzenie etniczne – HCL częściej dotyka mężczyzn pochodzenia aszkenazyjskiego niż mężczyzn z innych grup etnicznych2420

Choć HCL nie jest uważana za chorobę dziedziczną, istnieją doniesienia o rodzinnym występowaniu przypadków54. Analiza badań pacjentów z nowotworami krwi wykazała, że krewni pierwszego stopnia pacjentów z HCL mają zwiększone prawdopodobieństwo rozwoju tej choroby25, choć ryzyko pozostaje bardzo niskie26. Opisano przypadki HCL wśród członków rodziny z tym samym haplotypem HLA527.

Ważne jest jednak podkreślenie, że mutacja BRAF V600E nie jest anomalią przekazywaną przez rodziców – gen jest nieprawidłowy tylko w komórkach białaczkowych, a nie w komórkach prawidłowych2829. Naukowcy uważają, że mutacje genetyczne związane z chorobą nie są dziedziczone, ale raczej nabywane w ciągu życia2524.

Związek z innymi chorobami

U pacjentów z HCL zaobserwowano zwiększone ryzyko rozwoju innych typów nowotworów, w tym3013:

  • Chłoniak nieziarniczy (non-Hodgkin)
  • Chłoniak Hodgkina
  • Inne rodzaje nowotworów

Istnieje debata, czy zwiększone ryzyko jest związane z samą chorobą, czy z leczeniem stosowanym w HCL3013. Niektóre badania sugerują, że sama choroba może zwiększać to ryzyko13.

Ponadto, HCL może być powiązana ze schorzeniami autoimmunologicznymi31, choć związek ten wymaga dalszych badań.

Wnioski i perspektywy

Etiologia białaczki włochatokomórkowej jest wieloczynnikowa i obejmuje zarówno aspekty genetyczne, jak i środowiskowe32. Przełomowe odkrycie mutacji BRAF V600E jako prawdopodobnego czynnika wywołującego chorobę umożliwiło lepsze zrozumienie jej patogenezy oraz otworzyło drogę do nowych strategii terapeutycznych8.

Mimo tych postępów, wiele aspektów HCL pozostaje niejasnych, a badacze nadal poszukują dokładnych mechanizmów prowadzących do rozwoju tej rzadkiej choroby. Dalsze badania nad etiologią HCL mogą prowadzić do opracowania bardziej ukierunkowanych terapii, szczególnie dla pacjentów z wariantem HCL lub tych, którzy nie odpowiadają na standardowe leczenie8.

Warto podkreślić, że mimo przewlekłego charakteru choroby, przy odpowiednim leczeniu i opiece medycznej, pacjenci z HCL mogą mieć normalną długość życia3334. Głównym wyzwaniem pozostaje zrozumienie, co inicjuje zmiany genetyczne prowadzące do rozwoju HCL, co może przyczynić się do lepszych metod prewencji i leczenia w przyszłości.

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Hairy Cell Leukemia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK499845/
    The etiology of hairy cell leukemia is not well elucidated. However, previous exposure to various chemicals may play a role in its development. Most cases are postulated to be derived from V600E BRAF gene mutation of late activated memory B cells. […] HCL accounts for 2% of all leukemias with approximately 1000 new cases being reported in the United States each year.
  • #2 Hairy cell leukemia: short review, today’s recommendations and outlook | Blood Cancer Journal
    https://www.nature.com/articles/bcj20143
    Hairy cell leukemia (HCL) is part of the low-grade non-Hodgkin lymphoma family and represents approximately 2% of all leukemias. […] Recently, the identification of the possible disease-defining BRAF V600E mutation may allow the development of new therapeutic targets. […] The BRAF V600E mutation seems to have an important role in the pathogenesis of HCL if it is not even the disease-defining genetic event. […] The use of active BRAF inhibitors like BRAF V600E inhibitors could be an alternative to PA. […] These new insights of the pathogenesis of HCL may also allow new therapeutic options, especially for patients who need an alternative to PA because of non-sufficient response, persistence of BRAF-V600E at partial remission, relapse or toxic side effects of chemotherapy.
  • #3 Hairy Cell Leukemia: Practice Essentials, Pathophysiology, Epidemiology
    https://emedicine.medscape.com/article/200580-overview
    Hairy cell leukemia (HCL) is a chronic lymphoid leukemia, originally described in 1958 by Bouroncle and colleagues and named after the hairlike cytoplasmic projections seen on the surface of the abnormal B-cells. […] Hairy cell leukemia is recognized as a clonal B-cell malignancy, as identified by immunoglobulin gene rearrangements that result in a phenotype B-cell expression of surface antigens. These reflect the differentiation between the immature B-cell of chronic lymphocytic leukemia and the plasma cell of multiple myeloma. […] The etiology of HCL has not been determined, although some investigators suggest that exposures to benzene, organophosphorus insecticides, or other solvents may be related to disease development. This hypothesis has not been confirmed by other reports, although a French study that evaluated occupational exposure to pesticides and lymphoid neoplasms in men appears to support the hypothesis that occupational pesticide exposures may not only be involved in HCL, Hodgkin lymphoma, and multiple myeloma, but also may play a role in non-Hodgkin lymphoma.
  • #4 Clinical features and diagnosis of hairy cell leukemia – UpToDate
    https://www.uptodate.com/contents/clinical-features-and-diagnosis-of-hairy-cell-leukemia
    Hairy cell leukemia (HCL) is an uncommon indolent lymphoid malignancy characterized by the accumulation of neoplastic B cells with abundant cytoplasm and „hairy” projections within the peripheral blood, bone marrow, and splenic red pulp. HCL typically causes splenomegaly and variably reduced production of red blood cells, platelets, granulocytes, and monocytes. The resulting cytopenias have a variety of systemic consequences, including anemia, bleeding, and an increased risk of infection. […] The pathogenesis of HCL is incompletely understood. Most cases are postulated to arise from a late, activated memory B cell that acquires a BRAF V600E gene mutation. The resultant aberrant activation of the RAF-MEK-ERK signaling pathway leads to a distinct phenotype and enhanced cell survival. […] Exposures to ionizing radiation, pesticides, and farming have been mentioned as possible causes. Exposures to solvents, cigarette smoke, alcohol consumption, and obesity do not appear to be risk factors. Familial cases have been described. […] Cell of origin — HCL is postulated to arise from a late, activated memory B cell exhibiting preplasma cell or postgerminal center features.
  • #5 Hairy Cell Leukemia – Leukemia – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK586203/
    Hairy cell leukemia is a rare, indolent, chronic lymphoid neoplasm originating from a mature B lymphocyte. […] The pathogenesis of HCL is not fully understood. Exposure to ionizing radiation, pesticides, and farming have been suggested as possible etiologies, cases of HCL have been reported among family members with the same HLA haplotype. […] In most patients, HCL arises from a late, activated memory B cell that acquires a single somatic point mutation in the DNA sequence of v-Raf murine sarcoma viral oncogene homolog B (BRAF), a kinase-encoding proto-oncogene (BRAFV600E). […] The BRAFV600E mutation is detected in up to 80-90% of classic HCL patients. […] The mutation remains stable over the whole disease course, from the initial diagnosis to relapses, which can occur even many years after initial presentation.
  • #6 Hairy Cell Leukemia: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/23177-hairy-cell-leukemia
    Hairy cell leukemia is a rare type of blood cancer that happens when your bone marrow makes abnormal B-cells (B lymphocytes). […] Experts dont know the exact cause. Research shows that 90% of people with hairy cell leukemia have a genetic mutation (change) that affects the BRAF gene. This gene helps cell growth. When it changes, it creates abnormal cells that grow and multiply uncontrollably. This change happens after youre born. Researchers arent sure what triggers the change, but there are risk factors that increase your chance of developing hairy cell leukemia. […] Hairy cell leukemia risk factors include: Your age: This condition often affects people ages 40 to 70. On average, most of the people with hairy cell leukemia are 58 when they receive a diagnosis. Your sex: The condition affects men more than women. Exposure to Agent Orange: Researchers believe theres a link between this chemical and chronic B-cell leukemias and lymphomas, including hairy cell leukemia.
  • #7 Hairy cell leukemia – Wikipedia
    https://en.wikipedia.org/wiki/Hairy_cell_leukemia
    As with many cancers, the cause of HCL is unknown. Exposure to tobacco smoke, ionizing radiation, or industrial chemicals (with the possible exception of diesel) does not appear to increase the risk of developing it. […] Farming and gardening correlate with an increased risk of HCL development in some studies which does not necessarily imply causation. […] A 2011 study identified somatic BRAF V600E mutations in all 47 HCL patients studied, and no such mutations in the 193 peripheral B-cell lymphomas/leukemias other than HCL. […] The U.S. Institute of Medicine (IOM) found a correlation which permits an association between exposure to herbicides and later development of chronic B-cell leukemias and lymphomas in general. The IOM report emphasizes that neither animal nor human studies indicate an association of herbicides with HCL specifically. However, the IOM extrapolated data from chronic lymphocytic leukemia and non-Hodgkin lymphoma to conclude that HCL and other rare B-cell neoplasms may share this risk factor.
  • #8 Hairy cell leukemia: short review, today’s recommendations and outlook | Blood Cancer Journal
    https://www.nature.com/articles/bcj20143
    Hairy cell leukemia (HCL) is part of the low-grade non-Hodgkin lymphoma family and represents approximately 2% of all leukemias. […] Recently, the identification of the possible disease-defining BRAF V600E mutation may allow the development of new therapeutic targets. […] The BRAF V600E mutation seems to have an important role in the pathogenesis of HCL if it is not even the disease-defining genetic event. […] The use of active BRAF inhibitors like BRAF V600E inhibitors could be an alternative to PA. […] These new insights of the pathogenesis of HCL may also allow new therapeutic options, especially for patients who need an alternative to PA because of non-sufficient response, persistence of BRAF-V600E at partial remission, relapse or toxic side effects of chemotherapy.
  • #9 Hairy Cell Leukemia – Leukemia – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK586203/
    In addition to the BRAFV600E mutation, the most common genetic alteration in classical HCL is a loss in copy number for chromosome 7q. […] A mutated immunoglobulin heavy chain variable region (IGHV) gene profile is detected in 90% of HCL patients. […] The absence of a BRAF mutation (BRAFWT) is associated with the activation of mutations in the mitogen-activated protein kinase kinase 1 (MAP2K1) gene by unmutated IGHV and VH4-34 rearrangements; this small subset of classic HCL patients has poor prognosis and poor response to nucleoside purine analogs (PNAs).
  • #10 Hairy Cell Leukemia: Practice Essentials, Pathophysiology, Epidemiology
    https://emedicine.medscape.com/article/200580-overview
    Other suggested etiologic associations include exposure to radiation, agricultural chemicals, and wood dust, and a previous history of infectious mononucleosis. […] Overexpression of cyclin D1 protein, an important cell-cycle regulator, has been observed in HCL and may play a role in the molecular pathogenesis of the disease.
  • #11 Hairy Cell Leukemia-Variant: Causes, Diagnosis, and Treatment
    https://www.healthline.com/health/leukemia/hairy-cell-leukemia-variant
    Your bone marrow contains blood stem cells. These cells can turn into either a myeloid stem cell or a lymphoid stem cell. […] The exact causes of CLLs arent known, but experts believe that genetic changes called mutations can cause your B cells to multiply too often. […] Some of the known risk factors for CLL include: age over 50, exposure to radon or Agent Orange, close relatives (parents, siblings, or children) with CLL, race/ethnicity from North America or Europe. […] Classic HCL and HCL-V are genetically distinct. […] In classic HCL, theres usually a mutation on the BRAF V600E gene, but this mutation isnt there for people with HCL-V. Instead, HCL-V typically has a mutation on the MAP2K1 gene. […] For this reason, classic HCL treatments that target the mutated BRAF V600E gene dont work as a treatment for HCL-V.
  • #12 Hairy Cell Leukemia Pathogenesis, Diagnosis, and Prognosis
    https://www.onclive.com/view/hairy-cell-leukemia-pathogenesis-diagnosis-prognosis/1000
    Hairy cell leukemia (HCL) is an uncommon indolent B-cell lymphoid neoplasm. The cellular origin of HCL has long been debated. Phenotypically and morphologically, HCL tumor cells do not resemble any stages of normal B-cell maturation and development. Because of morphologic and functional similarities between hairy cells and monocytes/macrophages, the former were thought to have derived from a tumor cell from the reticuloendothelium. The discovery of rearrangement of the B-cell receptor immunoglobulin genes in patients with the disease demonstrated that HCL has a B-cell origin. Studies conducted with monoclonal antibodies have confirmed that hairy cells are mature B cells. The BRAF V600E mutation is a causal genetic event in the development of HCL. It is present in virtually all patients with classic HCL. Accumulating data suggest that the presence of BRAF V600E is the disease-causing event in HCL. This mutation is found in almost all cases of HCL, both at initial diagnosis and at relapse, regardless of the clinical presentation of the disease. In addition, the BRAF V600E mutation is detected in the entire tumor clone and is responsible for the hairy morphology, enhanced viability, and gene expression profile characteristic of HCL. Hence, inhibiting the effects of the BRAF V600E mutation appears to reverse the morphology and characteristics of hairy cells. […] In contrast to the classic form of HCL, BRAF V600E is not present in hairy cell leukemia variant (HCLv), a rarer form of B-cell neoplasm with a clinical course and response to therapy that are distinct from those of HCL.
  • #13 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation
    https://www.hairycellleukemia.org/hairy-cell-leukemia
    Hairy cell leukemia is a chronic and rare form of adult leukemia. It is estimated that approximately 2% of adult patients with leukemia have this form of disease. While the cause of this malignancy is not known, the origin of the malignant cell is thought to develop from the memory B cell compartment. […] While the cause is not known, the treatment is usually very effective in helping patients lead a normal life. […] There has been some concern that the therapy for hairy cell leukemia may increase the patients risk for developing a secondary cancer. This is an ongoing issue of debate. Some studies show that the disease itself may actually increase this risk. Therefore, people with HCL should continue to have their routine cancer screening performed at regular intervals based upon recommendations established by cancer experts. […] The variant form of hairy cell leukemia is now recognized as a separate clinical entity from the classic disease, and is recognized as being less responsive to therapy with standard treatment.
  • #14 Hairy Cell Leukemia > Fact Sheets > Yale Medicine
    https://www.yalemedicine.org/conditions/hairy-cell-leukemia
    Hairy cell leukemia is an uncommon type of cancer of the blood (leukemia) that affects the B cells, a type of white blood cell that makes antibodies to help the body fight infections. […] Doctors arent sure what causes hairy cell leukemia. It is sometimes linked to chemical exposure. Other people may have a genetic mutation that is linked to hairy cell leukemia. […] The most commonly associated genetic mutation is BRAF but another, CDKN1B, has also been identified in some cases. A gene mutation may cause someones B cells to mutate, become hairy and multiply uncontrollably, leading to cancer.
  • #15 Hairy cell leukemia – Wikipedia
    https://en.wikipedia.org/wiki/Hairy_cell_leukemia
    Human T-lymphotropic virus 2 (HTLV-2) has been isolated in a small number of patients with the variant form of HCL. In the 1980s, HTLV-2 was identified in a patient with a T-cell lymphoproliferative disease; this patient later developed HCL, but HTLV-2 was not found in the hairy cell clones. There is no evidence that HTLV-II causes any sort of hematological malignancy, including HCL. […] In a small population based study, there was an increased incidence of HCL in those who used or handled pesticides.
  • #16 Hairy Cell Leukemia: Symptoms, Diagnosis and Treatments
    https://www.healthline.com/health/hairy-cell-leukemia
    While the exact cause of HCL is unknown, some research shows a relationship between this type of cancer and exposure to the herbicide Agent Orange, which was used to destroy crops and jungle canopy during the Vietnam War. […] If you’re a veteran who has HCL and were exposed to Agent Orange during the Vietnam War, you may qualify for disability and healthcare benefits from the United States Department of Veterans Affairs.
  • #17 Causes and Risk Factors of Hairy Cell Leukemia
    https://www.medindia.net/health/conditions/causes-and-risk-factors-of-hairy-cell-leukemia.htm
    Hairy cell leukemia appears to have a genetic background. Some environmental factors may also increase the risk of suffering from the cancer. […] The exact cause of hairy cell leukemia is unknown; but it appears to have a genetic background. Hairy cell leukemia has been associated with mutations in the BRAF gene and over expression of cyclin D1 protein. […] Conditions that possibly increase risk of developing hairy cell leukemia include: Exposure to high dose ionizing radiations […] Long term exposure to organic solvents such as benzene, other aromatic solvents, petroleum products and agricultural pesticides […] Exposure to wood and sawdust […] Family history of hairy cell leukemia or any other malignant blood disorders […] Presence of non-malignant disorders such as myelofibrosis, bone marrow aplasia, amyloidosis and granulomatous diseases.
  • #18 Hairy cell leukemia
    https://www.mymlc.com/health-information/diseases-and-conditions/h/hairy-cell-leukemia2/
    Hairy cell leukemia is considered a chronic disease because it may never completely disappear, although treatment can lead to a remission for years. […] It’s not clear what causes hairy cell leukemia. […] Doctors know that cancer occurs when cells develop errors (mutations) in their DNA. In the case of hairy cell leukemia, mutations in the DNA cause your bone marrow stem cells to create too many white blood cells that don’t work properly. Doctors don’t know what causes the DNA mutations that lead to hairy cell leukemia. […] Certain factors may increase your risk of developing hairy cell leukemia. Not all research studies agree on what factors increase your risk of the disease. […] Some research indicates that your risk of hairy cell leukemia increases based on your exposure to radiation. People exposed to radiation, such as those who work around X-ray machines and do not wear adequate protective equipment or those who received radiation treatment for cancer, may have a higher risk of developing hairy cell leukemia, but the evidence is inconclusive. […] There are conflicting studies on the role of industrial and agricultural chemicals in hairy cell leukemia development.
  • #19 Hairy cell leukemia – CheckOrphan
    https://checkorphan.org/disease/hairy-cell-leukemia/
    Hairy cell leukemia is a rare, slow-growing cancer of the blood in which the bone marrow produces too many B cells (lymphocytes), a type of white blood cell that fights infection. […] The underlying cause of this condition is unknown. Cancers are caused by a defect in your DNA. In the case of hairy cell leukemia, mutations in the DNA cause your bone marrow stem cells to create too many white blood cells that don’t work properly. It is not clear what causes the DNA mutations that lead to hairy cell leukemia. […] Certain factors may increase your risk of developing hairy cell leukemia. Not all research studies agree on what factors increase your risk of the disease. […] Some research indicates that your risk of hairy cell leukemia increases based on your exposure to radiation. People exposed to radiation, such as those who work around X-ray machines or those who received radiation treatment for cancer, may have a higher risk of developing hairy cell leukemia, but the evidence is inconclusive.
  • #20 Hairy cell leukemia – CheckOrphan
    https://checkorphan.org/disease/hairy-cell-leukemia/
    Exposure to chemicals. Industrial and agricultural chemicals could play a role in hairy cell leukemia development. However, some studies have found this not to be the case. […] Exposure to sawdust. Some studies have found a link between working with wood and sawdust and an increased risk of hairy cell leukemia. But this connection hasn’t been proved conclusively. […] Ethnicity. Hairy cell leukemia affects men of Ashkenazi Jewish ancestry more frequently than men of other ethnic groups.
  • #21 Hairy cell leukemia, an uncommon B-cell lymphoid neoplasia | Medicina Universitaria
    https://www.elsevier.es/en-revista-medicina-universitaria-304-articulo-hairy-cell-leukemia-an-uncommon-S1665579616300151
    Hairy cell leukemia (HCL) is an uncommon B-cell lymphoid neoplasia, representing 23% of all leukemias. […] Although its etiology has not been established, the recent gene sequencing of HCL identified the presence of the BRAF V600E mutation, absent in other malignant neoplasias of the lymph cells. […] Although there are different studies showing a link between exposure to certain agents and HCL, its etiology has not been clearly established, which could be explained due to its low incidence. Amongst the agents which have proven a positive link are: exposition to pesticides, herbicides, mineral oils, working as a carpenter, or a farmer. […] Recently, a positive link with size has been described, whereas smoking has an inverse association, especially in men. […] The specific mechanism which confers the protection is unknown; nevertheless, smoking has been suggested to decrease the severity of inflammatory mechanisms and that nicotine induces apoptosis in lymphocytes.
  • #22 Hairy cell leukemia
    https://sales-demo.adam.com/content.aspx?productid=141&pid=1&gid=000592
    Hairy cell leukemia (HCL) is an unusual cancer of the blood. It affects B lymphocyte cells, a type of white blood cell. […] HCL is caused by the abnormal growth of certain B cells. The cells look „hairy” under the microscope because they have fine projections extending from their surface. […] The cause of this disease is unknown. Certain genetic changes in the cancer cells may be the cause. It affects men more often than women. The average age of diagnosis is 55. […] There is no known way to prevent this disease.
  • #23 What Is Hairy Cell Leukemia? Treatment, Symptoms, and Causes
    https://www.everydayhealth.com/hairy-cell-leukemia/
    As with other cancers, researchers have linked hairy cell leukemia to changes (mutations) in the DNA of cells. […] Per a research review, scientists now understand that the vast majority of people with hairy cell leukemia have a mutation in the BRAF gene a gene also implicated in a number of other cancers, including melanoma. […] Scientists are still working to understand what causes the BRAF mutation, and hairy cell leukemia in general. […] They have investigated possible risk factors, including exposure to industrial or agriculture chemicals or radiation (which may affect people in occupations that involve X-rays). But studies so far have been inconclusive or have resulted in conflicting findings. […] HCL is 4 times more common in males than females, and usually presents at age 50 or above.
  • #24 Hairy Cell Leukemia — Leukemia Research Foundation
    https://leukemiarf.org/leukemia/chronic-lymphocytic-leukemia/hairy-cell-leukemia/
    Most people (95%) with HCL have a mutation (change) in the V600E BRAF gene. This change allows B-cells to divide rapidly, survive, and become cancerous without control. […] HCL is not passed down from your parents. Instead, these mutations occur during a persons lifetime. […] Risk factors for HCL include: Agent Orange exposure, Ashkenazi Jewish ancestry, Family history of HCL (This does not mean that you inherit HCL from your parents. Instead, the risk of developing the same type of genetic mutation after birth slightly increases.), Large-animal farming, Middle-aged men (four times as likely than in women), Pesticide/chemical exposures, White ethnicity.
  • #25 What Is Hairy Cell Leukemia? Treatment, Symptoms, and Causes
    https://www.everydayhealth.com/hairy-cell-leukemia/
    Hairy cell leukemia is not generally considered to be an inherited disorder. […] However, a research analysis of patients with blood cancers showed that first-degree relatives of hairy cell leukemia patients have an increased probability of developing hairy cell leukemia. […] Scientists believe that the genetic mutations behind the disease arent passed down by ones parents but rather acquired during ones lifetime.
  • #26 Risks and causes of hairy cell leukaemia | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/hairy-cell-leukaemia/risks-causes
    We don’t know what causes hairy cell leukaemia. But there are some factors that may increase your risk of developing it. […] Your risk is slightly higher if you have a close relative (parent, sibling, child) diagnosed with hairy cell leukaemia. But most people with a family history of hairy cell leukaemia do not develop it themselves and the risk remains very low. […] Researchers around the world are looking into the causes and treatment of hairy cell leukaemia.
  • #27 Hairy Cell Leukemia | Concise Medical Knowledge
    https://www.lecturio.com/concepts/hairy-cell-leukemia/
    Hairy cell leukemia is a clonal B-cell malignancy. The exact etiology is unknown, but several environmental exposures appear to increase the risk of developing HCL. […] Exposures as possible causes include ionizing radiation, organophosphorus pesticides, farming and agricultural chemicals, wood dust, and previous history of infectious mononucleosis. […] Familial relationships are due to sharing an HLA haplotype.
  • #28 Hairy Cell Leukaemia (HCL) | Leukaemia Care
    https://www.leukaemiacare.org.uk/support-and-information/information-about-blood-cancer/blood-cancer-information/leukaemia/hairy-cell-leukaemia/
    The cause of HCL is not known. The only factors which are definitely known to increase the chance of developing the condition are older age and being male. […] A very important recent discovery has been the fact that a very high proportion, possibly all, hairy cells have a specific mutation of a gene called BRAF. This is not an abnormality which has been passed on by parents; the gene is only abnormal in your hairy cells and not in your normal cells.
  • #29 What is hairy cell leukaemia? | Blood Cancer UK
    https://bloodcancer.org.uk/understanding-blood-cancer/leukaemia/hairy-cell-leukaemia/what-is-hairy-cell-leukaemia/
    Hairy cell leukaemia is a type of blood cancer that develops slowly. It affects white blood cells called lymphocytes, which protect you from infection. […] Hairy cell leukaemia is a type of blood cancer that affects cells called B lymphocytes. These are a type of white blood cell. They are part of the immune system and help your body to fight infections. […] In hairy cell leukaemia, your bone marrow makes abnormal B lymphocytes. They dont work properly, so they cant protect you from infection like they should. […] Its not clear what causes hairy cell leukaemia. Hairy cell leukaemia can happen to anyone, and its not because of anything you have done. […] There is no clear evidence to suggest that hairy cell leukaemia runs in families. […] People with a close relative with hairy cell leukaemia (a parent, sibling or child) have a slightly increased risk of developing it themselves. But overall the risk is still very low, and most people with a family history never develop hairy cell leukaemia. […] Recent research has found that the cancer cells in hairy cell leukaemia have a specific mutation of the BRAF gene. But this is a mutation that happens when hairy cell leukaemia develops its not a mutation found in healthy cells in the body, so its not something that can be passed down to children.
  • #30 Hairy cell leukemia – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/hairy-cell-leukemia/symptoms-causes/syc-20372956
    It’s not clear what causes hairy cell leukemia. […] Hairy cell leukemia happens when B cells develop changes in their DNA. A cell’s DNA contains the instructions that tell a cell what to do. The changes tell the B cells to make a lot more B cells that don’t work right. […] Some studies found that people with hairy cell leukemia have an increased risk of other types of cancer. The other cancers include non-Hodgkin’s lymphoma, Hodgkin’s lymphoma and others. It’s not clear if the other cancers are caused by hairy cell leukemia or by cancer treatments.
  • #31
    https://step2.medbullets.com/oncology/120475/hairy-cell-leukemia-hcl
    ETIOLOGY […] Associated conditions […] autoimmune conditions […] BRAF V600E mutation
  • #32 Pathology Outlines – Hairy cell leukemia
    https://www.pathologyoutlines.com/topic/lymphomahcl.html
    Hairy cell leukemia (HCL) is a rare, indolent lymphoproliferative neoplasm of mature B cells with a distinct clinical presentation that includes peripheral blood cytopenias, splenomegaly and a small number of circulating neoplastic cells with hair-like cytoplasmic projections […] Epidemiologic data shows a multifactorial etiology that is influenced by ethnicity and geographical factors […] Farming and exposure to pesticides, petroleum products, diesel and ionizing radiation are associated with an increased risk.
  • #33 Hairy cell leukemia – Associates in Obstetrics and Gynecology, P.C.
    https://associates-obgyn.com/patient-education/healthwise?DOCHWID=bc1140
    Hairy cell leukemia is a very rare cancer that causes the body to make too many white blood cells (lymphocytes). These lymphocytes don’t develop normally and become leukemia cells. Hairy cell leukemia has this name because the leukemia cells look hairy under a microscope. […] HCL may grow very slowly or it may not get worse. So it often does not need to be treated right away. […] HCL doesn’t ever go away completely. But treatment can keep symptoms away for long periods of time. When under the care of a doctor, a person who has HCL can have a normal life expectancy.
  • #34 Hairy Cell Leukemia (HCL): Causes, Symptoms & Diagnosis
    https://myhematology.com/white-blood-cells/hairy-cell-leukemia/
    Identifying specific risk factors for hairy cell leukemia (HCL) is challenging. While the exact cause remains unknown, certain factors may increase the risk: Age: The incidence of HCL increases with age, with the peak occurring in the sixth and seventh decades of life. […] The prognosis for patients with HCL has significantly improved due to advancements in treatment. Overall, the outlook for patients with HCL is good. Long-term survival is common and many patients can live normal lives with effective treatment. […] Yes, hairy cell leukemia (HCL) can spread to other organs. While the spleen is the most commonly affected organ, HCL can also involve the liver, bone marrow, and lymph nodes. […] Bone marrow biopsy is the confirmatory test for hairy cell leukemia (HCL). It involves removing a sample of bone marrow tissue to examine under a microscope. The presence of hairy cells, which are abnormal B cells with characteristic hair-like projections, is a hallmark of HCL. […] Yes, it is possible to survive hairy cell leukemia. With advancements in treatment, many people with HCL can live long and productive lives. The prognosis for HCL has significantly improved in recent years, and many patients can achieve long-term remission.