Materiały źródłowe

• #1 Hairy Cell Leukemia – Leukemia – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK586203/

  Hairy cell leukemia accounts for less than 2% of all leukemias. Its incidence is 0.3 cases per 100,000 individuals with an average male-to-female ratio of 1.5-2:1 and median age at diagnosis of 58 years. The incidence is approximately three times higher in White than in Black populations. HCL-v is estimated to be 0.2 cases per 100,000. HCL-v affects mainly elderly patients with a median age of 71 years.

• #2 Hairy Cell Leukemia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/22488

  Hairy cell leukemia is a rare neoplasm representing 2% of lymphoid leukemias. Median age at diagnosis is 55 years old. Although it may occur in younger individuals, it is almost never seen in children. Males are disproportionately affected compared to females, with a ratio of 4:1. […] HCL accounts for 2% of all leukemias with approximately 1000 new cases being reported in the United States each year.

• #3 Orphanet: Classic hairy cell leukemia

  https://www.orpha.net/en/disease/detail/58017

  Classic hairy cell leukemia (HCLc) accounts for 2% of all leukemia cases. Estimates of the annual incidence range between 1/213,000-2,860,000 worldwide. HCLc is observed more commonly in caucasians. Men are predominantly affected with a male:female ratio of 4:1. […] Disease onset is typically in middle-age or older adults with an average age of 55 years. […] Most patients live 10 years or longer with the disease.

• #4 Hairy Cell Leukemia: A Review of Epidemiology and Disease Presentation

  https://www.onclive.com/view/hairy-cell-leukemia-a-review-of-epidemiology-and-disease-presentation

  Hairy cell leukemia (HCL) is a rare lymphoid malignancy. HCL accounts for only 2% of all lymphoid leukemias, with 600 to 800 new cases diagnosed in the United States each year. […] HCL is a rare hematologic neoplasm. According to 1992 to 2001 data, approximately 1000 new cases of HCL are diagnosed each year in the United States, HCL accounts for 2% of all leukemias and 1% of all lymphoid neoplasms in the United States. […] The male predominance of HCL has been well documented, with a male-to-female ratio of about 4:1. Data suggest that in the United States, the median age at diagnosis is 50 years (range, 28 to 79 years). […] The incidence of HCL varies according to ethnicity and genetic background. The disease is less common among individuals of Asian, African, and Arab descent, and it is more common in Caucasians, particularly among Jewish individuals.

• #5 Hairy Cell Leukemia: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/200580-overview

  Hairy cell leukemia is relatively uncommon, accounting for 2% of all leukemia cases. An estimated 1100 new cases were diagnosed in the United States in 2016. Hairy cell leukemia is observed more commonly in whites. It has been reported to occur in Ashkenazi Jews, and it is rare in Japan and in people of African descent. […] The disease occurs predominantly in middle-aged men, with a median age of 49-51 years. The male-to-female ratio is 4:1 to 5:1.

• #6 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. […] There are approximately 1,000 new cases of hairy cell leukemia discovered every year in the United States. There is an unexplained larger number of men than women afflicted with this leukemia. […] Hairy cell leukemia variant is a rare disorder accounting for approximately 0.4% of chronic lymphoid malignancies and 10% of all HCL cases. The disease affects the elderly population without sexual predominance. The median age of the patients is 71 years.

• #7 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia.com/en/onkopedia/guidelines/hairy-cell-leukemia

  HCL is rare with an incidence of about 0.3-0.4/100,000 persons (crude disease rate). In Germany, approximately 70 women and 220 men develop the disease annually. Incidence and distribution are concordant with data from other countries. The disease accounts for about 3% of lymphocytic leukemias in the current evaluation. The median age of onset in Germany was most recently between 62 (men) and 65 (women) years. First diagnoses before the age of 30 are very rare, men are affected significantly more often than women in all age groups. […] In the last decade, a trend towards a somewhat earlier age of onset of disease has been seen in the centers. This is presumably a consequence of the greater use of general screening measures by younger people, with performance of a blood count. As a result, cytopenias are diagnosed earlier, i.e. before the onset of clinical symptoms.

• #8 Hairy Cell Leukemia Epidemiology Forecast and Emerging Therapies

  https://www.delveinsight.com/blog/hairy-cell-leukemia-therapy-landscape

  In the U.S., approximately 6,000 persons get affected by Hairy cell leukaemia and around 600-800 new cases are diagnosed each year. Hairy cell leukaemia affects males four times more often than females (M4: F1), as observed by Rare diseases organization. The mostly male population of age 50 and older are more prone to developing Hairy cell leukaemia. However, the Hairy Cell leukaemia occurs in individuals between 20 to 80 years of age. […] Literature suggests that most patients survive for ten years or longer with the disease. As per the Cancer Research U.K., around 90% of the patients will survive for five years or more after the diagnosis. DelveInsight estimates that the total Hairy Cell Leukemia incident population in the 7MM was 2,364 in 2017. Out of which, there were 755 diagnosed incident HCL cases reported in the U.S.

• #9 Hairy Cell Leukemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23177-hairy-cell-leukemia

  Hairy cell leukemia is a rare condition that affects 1 in 100,000 people in the U.S. […] Its more common in people who are white than people in other ethnic groups. […] Hairy cell leukemia grows very slowly. You may have it for years before developing symptoms. […] If you dont have symptoms, your oncologist may recommend delaying treatment until you develop symptoms. […] In active surveillance, youll see your oncologist every three to six months. […] Most people need medical care and support for the rest of their lives so their providers can watch for recurrent hairy cell leukemia. […] If you have this disease but dont have symptoms, youll have checkups and tests every three to six months after your diagnosis. […] According to the Leukemia and Lymphoma Society, 90% of people treated with the chemotherapy drug cladribine were alive five years after treatment.

• #10 Hairy cell leukemia | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/hairy-cell-leukaemia?embed_domain=external.radpair.com%27%5B0%5D%27%5B0%5Dradiopaedia-icon-144.pngfavicon.icofavicon.ico&lang=us

  Its annual incidence is estimated at around 0.3 cases per 100 000, and the disease comprises 2-3% of all leukaemias. There is a recognized male predilection of around 4:1 with a median age of around 55 years. […] Hairy cell leukemia is a rare and indolent form of small mature B-cell leukaemias.

• #11 Introduction and Epidemiology of Hairy Cell Leukemia

  https://www.onclive.com/view/introduction-and-epidemiology-of-hairy-cell-leukemia

  When it occurs in younger patients, it tends to be somewhat more aggressive. The prognosis in younger people isn’t as good as in older people, but older patients can also die of unrelated causes. It’s probably a little bit more biologically active in younger patients. […] About 60% to 80% of people are found to have pancytopenia, and neutropenia particularly, as well as monocytopenia. They’re both in the range of 80% of patients who will have this disorder. […] One of the very profound features would be splenomegaly. Up to 90% or more of patients have splenomegaly. […] What’s interesting with hairy cell compared to other leukemias and lymphomas is it’s much less common to have lymph nodes involved. Clear splenomegaly is a clue that hairy cell is in the differential, among other things as well.

• #12 Introduction and Epidemiology of Hairy Cell Leukemia

  https://www.onclive.com/view/introduction-and-epidemiology-of-hairy-cell-leukemia

  Hairy cell leukemia is a relatively uncommon hematologic malignancy. There are about 600 to 800 cases annually in the United States. I think that’s underestimated. That was before the introduction of flow cytometry, where it’s easier to detect hairy cell leukemia circulating in the peripheral blood. It’s a disease of males rather than females. The ratios about 4:1, usually middle-aged patients, but theres great variability. The youngest patient Ive seen is 26, and I’ve seen the disease in 90-year-olds. It’s mostly a disease of Caucasians. Why it doesn’t affect other ethnic groups to the same degree Im uncertain, but it certainly occurs in Hispanics. Of the 800 patients I’ve seen, probably only 1 has been African American, and I’ve seen a handful of Asian Americans with this disorder. […] Among the Caucasians, theres a higher incidence among Ashkenazi Jews. The reason for that is unclear. It is a disorder of the memory pre-plasma cell, and circulating lymphocytes can accumulate in the spleen, less commonly in lymphocytes in the bone marrow, and hence the clinical presentation of splenomegaly, cytopenias. Monocytopenia is a hallmark of untreated hairy cell leukemia. This is a disease with a very indolent and protracted clinical course, and we’ll get into the various factors associated with it.

• #13 Hairy Cell Leukemia: Retrospective Analysis of Demographic Data and Outcome of 203 Patients from 12 Medical Centers in Israel | Anticancer Research

  https://ar.iiarjournals.org/content/38/11/6423

  Hairy cell leukemia (HCL) is an uncommon indolent B-cell lymphoproliferative disorder occurring in 3/1,000,000 patients (1). […] The median age of diagnosis was 55.5 years for Jews and 49 years for Arabs (p=0.021), and most patients were males (81.77%); 52.2% were Ashkenazi Jews, 36.1% Sephardic Jews and 11.7% were Arab, Druze or other ethnicity. […] It is of interest that Arab patients with HCL were diagnosed at an earlier age, but had a similar clinical course and outcome to both Ashkenazi and Sephardic Jews. […] The Israeli population is heterogeneous and multi-ethnic, with a 75% Jewish majority, consisting of new immigrants and Israeli-born Jews. The non-Jewish (mostly Muslim Arab) minority represents approximately 25% of the entire population. […] There are only sparse data relating to the epidemiology of HCL and on prognosis according to different ethnic groups, and it is in this respect the Israeli patient cohort is of interest.

• #14 Hairy Cell Leukemia: Retrospective Analysis of Demographic Data and Outcome of 203 Patients from 12 Medical Centers in Israel | Anticancer Research

  https://ar.iiarjournals.org/content/38/11/6423

  HCL was also more frequent in Jews (88% of the entire cohort), which is similar to that encountered in chronic lymphocytic leukemia, which is also more common in Ashkenazi Jews (23-25). […] In summary, we herein report variations in the incidence and prognosis of HCL in Israel, mostly related to gender, age and ethnicity. HCL is less frequently encountered in the Arab population, where it occurs at a younger age, and is more common among Ashkenazi than Sephardic Jews. Additional genetic or genomic-wide association studies are still needed to support the observations reported here regarding ethnicity.

• #15 Orphanet: Hairy cell leukemia variant

  https://www.orpha.net/en/disease/detail/300878

  Hairy cell leukemia variant (HCL-V) constitutes about 10% to 20% of patients with hairy cell leukemia (HCL) and 0.4% of all lymphoid malignancies. There is a slight male predominance (male/female: 1.6). […] HCL-V onset is in adulthood, most frequently in the elderly population (median age: 71 years). […] HCL-V is more aggressive and resistant to therapy than classical HCL (HCL-C). […] Diagnosis is suspected on physical examination, bone marrow biopsy, and abdominal computed tomography (CT) scan, as well as laboratory tests which show raised white blood cell count (10109/l). […] Asymptomatic patients with moderate splenomegaly and no cytopenias may be managed with close monitoring. […] HCL-V is an aggressive disorder with a chronic clinical course. The reported survival at five years is approximately 60%; however, complete remission has been reported, in particular with combined purine analog and rituximab treatment or immunotherapy alone.

• #16 Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group

  https://www.mdpi.com/2072-6694/16/12/2185

  HCL accounts for 2–3% of all leukemias and fewer than 1% of all hematological disorders. […] The incidence rate was 0.3 per 100,000 for HCL and 0.2 per 100,000 for HCL-V in the United States. It was lower in non-Hispanic/Blacks, Hispanics, and Asians/Pacific Islanders. In 2018, the number of new cases was estimated at 300 in France. Incidence between 1990 and 2018 remained relatively stable despite the aging of the population and better access to diagnostic tools. […] The male-to-female sex ratio is 5:1. […] At diagnosis, the median age is 63 years for men and 59 years for women. […] The etiology of HCL remains largely unknown but several risk factors have been suspected: farming, exposure to pesticides, petroleum derivatives, diesel and ionizing radiation. Tobacco appears to be protective for HCL. Rare familial cases have also been described.

• #17 Hairy Cell Leukemia: A Review of Epidemiology and Disease Presentation

  https://www.onclive.com/view/hairy-cell-leukemia-a-review-of-epidemiology-and-disease-presentation

  Although several large studies have attempted to elucidate occupational, lifestyle, and environmental risk factors associated with HCL, the definitive risk factors have not yet been identified. […] Data suggest that cigarette smoking may be inversely associated with HCL. The frequency and duration of cigarette smoking, as well as lifetime cigarette exposure, were all significantly associated with a reduced risk for the disease. […] There have been rare anecdotal case reports of familial HCL. Although in some cases of familial HCL, affected family members have been reported to share human leukocyte antigen (HLA) haplotypes, a specific HLA antigen or haplotype common to most cases of familial HCL has not been identified.

• #18

  https://www.msjonline.org/index.php/ijrms/article/view/2684

  Hairy cell leukemia (HCL) is a rare chronic B-cell leukemia accounting for 2% of all the leukemias and occurs more frequently in the elderly males. […] The etiology is unknown but possible relationships to radiation exposure, exposure to benzene, to farm animals and to commercial herbicides and pesticides have been identified. […] Familial predisposition among first degree relatives has been noted. […] We report a rare case of 29 year old female, farm labourer presenting with fever, fatigue and weakness for 1 month.

• #19 Hairy cell leukemia (Chapter 7) – Management of Hematologic Malignancies

  https://www.cambridge.org/core/books/management-of-hematologic-malignancies/hairy-cell-leukemia/E6C4592036823911BB563F26152CF566

  Hairy cell leukemia (HCL) is a rare, chronic lymphoproliferative disorder characterized by splenomegaly, pancytopenia, bone marrow fibrosis, and frequent infectious complications. HCL constitutes approximately 2% of all lymphoid leukemias. It is predominantly a male disease, with the male:female ratio ranging from 4:1 to 7:1. The vast majority of affected people are white, with Ashkenazi Jews being an overrepresented group. The median age of onset is in the early fifth decade. […] HCL has no known cause and risk factors are poorly characterized. Farming or woodworking, or occupational exposure to organic solvents, resulted in a higher relative risk for developing HCL. Infectious mononucleosis has been associated with HCL, but a pathogenic role for EpsteinBarr virus has been disputed. Studies evaluating radiation exposure as a HCL risk factor have also produced conflicting results.

• #20 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. […] Incidence: < 2% of all leukemias, < 1 per 100,000 persons/year in U.S. [...] M:F = 4:1. [...] Median age of diagnosis is 55 years. [...] More common in whites and less in African, Asian and Arab populations. [...] Increased risk: farming and exposure to pesticides (Scand J Work Environ Health 1996;22:285). [...] Smoking has been reported as a significant negative association and appears to be protective (Br J Haematol 1995;91:154).

• #21 Hairy cell leukemia – Wikipedia

  https://en.wikipedia.org/wiki/Hairy_cell_leukemia

  HCL is more common in men, with the male to female distribution is 4 to 1, and the median age at diagnosis is age 55-60. […] The incidence in the United States and Europe is 0.28-0.3 cases per 100,000, and the prevalence is 3 cases per 100,000 people in Europe. It is less common in Africa, the Middle East and Asia. […] It does not appear to be hereditary, although rare familial cases that suggest a predisposition have been reported, usually showing a common Human Leukocyte Antigen (HLA) type.

• #22 Hairy Cell Leukemia (HCL) | Flow Cytometry

  https://wiki.clinicalflow.com/hairy-cell-leukemia-hcl

  This disease is rare, with fewer than 1 in 10,000 people being diagnosed with HCL during their lives. […] Although most patients are white males over the age of 50, it has been diagnosed in at least one teenager. […] Men are four to five times more likely to develop hairy cell leukemia than women. […] It does not appear to be hereditary, although occasional familial cases have been reported, usually showing a common HLA type.

• #23 Autoimmune conditions and hairy cell leukemia: an exploratory case-control study | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/1756-8722-3-35

  Case reports suggest that hairy cell leukemia (HCL) may be associated with autoimmune conditions, however no systematic investigations in this area have been undertaken. […] Using the United States Surveillance, Epidemiology, and End Results Medicare linked database, we conducted an exploratory study comparing autoimmune conditions in 418 HCL cases (aged 65 years) and 160,086 controls. […] Overall, the proportion with autoimmune conditions was similar between HCL cases and controls (n = 79 (18.9%) and n = 29,284 (18.3%), respectively). […] Before diagnosis/selection, there was no overall difference in the prevalence of autoimmune conditions in HCL cases (n = 37, 8.9%) compared with controls (n = 14,085, 8.8%), p = 0.969. […] However, compared with controls, HCL cases more frequently had sarcoidosis (OR 9.6, 95%CI 2.4-39.5), Sjgren syndrome (OR 6.1, 95%CI 2.0-19.3) and erythema nodosum (OR 37, 95%CI 4.9-284) before diagnosis.

• #24 Autoimmune conditions and hairy cell leukemia: an exploratory case-control study | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/1756-8722-3-35

  Autoimmune conditions were also more common in HCL cases than controls around the time of diagnosis/selection (p 0.001) but not subsequently. […] The findings do not support an overall relationship between autoimmune conditions and HCL, although the association with some autoimmune conditions prior to HCL diagnosis may warrant further investigation. […] Our findings also suggest that autoimmune conditions in HCL patients may be detected around the time of diagnosis. […] In conclusion, our study found little difference in the occurrence of autoimmune conditions between HCL cases and controls except at the time around HCL diagnosis. […] Despite small numbers of affected individuals, sarcoidosis, Sjgren syndrome and erythema nodosum were associated with an increased risk of subsequent HCL.

• #25 Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up | Blood Cancer Journal

  https://www.nature.com/articles/s41408-020-0328-z

  In total, 279 patients with hairy-cell leukemia (HCL) were analyzed, with a median follow-up of 10 years. […] HCL patients require long-term follow-up because of their relatively increased risk of second malignancies. […] The risk of second malignancies occurring during follow-up in HCL patients is controversial, with some studies describing a higher risk of cancer than in the general population and others describing no increased risk. […] In our cohort, we found that second cancers were the primary cause of death. […] Our study also showed that patients with HCL had a risk of developing second cancers. This risk was higher than in the general population: the SIRs for all cancers, solid tumors, and hematological malignancies were 2.22, 1.81, and 6.67, respectively. […] In multivariate analysis, IFN was a protective factor against cancer, while a personal history of cancer was a risk factor for hematological malignancies. […] In our study, we confirmed the favorable prognosis of HCL. PNAs are the best choice of treatment and result in the best CR rate, DOR, RFS, and CIR. Relapses are still an issue, with a 10-year CIR of 39%.

• #26 Treating Hairy Cell Leukemia | American Cancer Society

  https://www.cancer.org/cancer/types/chronic-lymphocytic-leukemia/treating/hairy-cell-leukemia.html

  Hairy cell leukemia (HCL) tends to grow slowly. […] People with HCL who aren’t having symptoms often don’t need to be treated right away, but they do need to be watched carefully. […] If you have HCL, you will see your cancer care team every few months to check for signs that the HCL is growing and to see if it’s causing any problems (like low blood counts, fatigue, or an enlarged spleen). […] Some people with HCL live for many years without having symptoms or needing treatment. […] Treatment may be advised if you have low blood cell counts, recurrent infections, an enlarged spleen or lymph nodes, or other bothersome symptoms. […] Treatment doesn’t cure HCL, but it can help with symptoms, stop the HCL from progressing, and help you live longer. […] HCL usually responds well to these drugs, and the responses often last for many years.

• #27 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia-guidelines.info/en/onkopedia/guidelines/hairy-cell-leukemia

  Survival is better than in CLL: 10 years after initial diagnosis, survival compared to the age-matched general population (relative survival) is 90% (CLL: 72%). […] A worsening of life expectancy is particularly evident in the first year after diagnosis, after which relative life expectancy is even above average. In other evaluations, too, patients with HCL have a life expectancy after therapy with purine analogs that corresponds approximately to the normal population. […] There is no evidence of effective interventions for prevention and early detection. […] HCL is a chronic disease. Late relapses are possible. There is no prospectively evaluated control program. A risk-adapted approach is recommended: In the first 6 months after achieving optimal response, blood count checks every 4 weeks are reasonable, abdominal sonography to check spleen size every 3 months. In case of stable hematologic remission, the examination intervals for blood counts can be extended to 3 months or for sonography to 6 months or more. Blood count changes again suggest shorter follow-up intervals. A suitable complementary parameter is the determination of soluble interleukin-2 receptor in peripheral blood. Bone marrow biopsies are usually not required. […] Patients with HCL should participate in the recognized measures of prevention and early detection of cancer offered by the health insurances.

• #28 Hairy Cell Leukemia > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/hairy-cell-leukemia

  Hairy cell leukemia is a rare form of cancer, comprising only about 2% of leukemia cases in adults each year. About 700 people are diagnosed with hairy cell leukemia annually. It is five times more common in men than in women and the vast majority (90%) of patients are of European ancestry. People are typically diagnosed during middle age, but it is occasionally found in people in their 20s, as well as in older adults. […] Because this form of cancer responds well to treatment, more than 95% of people who are diagnosed survive 10 years or longer. The majority of people experience complete remission after treatment. […] Although 30 to 40% of people who go into remission may experience relapse, the treatments for hairy cell leukemia are so effective, people are often able to lead normal lives between diagnoses.

• #29 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia.com/en/onkopedia/guidelines/hairy-cell-leukemia

  There is evidence that patients with HCL have an increased risk of second malignancies. The published data from long-term observations diverge. A significantly increased rate of hematologic neoplasms has been described. The pattern of secondary solid tumors is not substantially different from malignancies occurring in men and women aged 60 years.

• #30

  https://link.springer.com/article/10.1007/s00277-020-04349-z

  Skin lesions have been reported in about 10-12% of hairy cell leukemia (HCL) patients. […] This paper reviews the epidemiology, pathogenesis, clinical symptoms, diagnosis, and approach to treating skin lesions in HCL. […] The annual incidence is between 2.9 and 4.7 per million people per year. […] Cutaneous symptoms have been reported in 10-12% of HCL patients. […] The direct infiltration of the skin (i.e., leukemia cutis) is extremely rare in HCL patients. […] The overall incidence of second primary tumors in patients with HCL ranges from 19.9 to 24%. […] Existing data suggests that patients with HCL have a higher risk of non-melanoma skin cancers; therefore, these patients should be closely examined, especially when secondary risk factors exist like fair skin and extensive sun burden.

• #31 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia.com/en/onkopedia/guidelines/hairy-cell-leukemia

  Survival is better than in CLL: 10 years after initial diagnosis, survival compared to the age-matched general population (relative survival) is 90% (CLL: 72%). […] A worsening of life expectancy is particularly evident in the first year after diagnosis, after which relative life expectancy is even above average. In other evaluations, too, patients with HCL have a life expectancy after therapy with purine analogs that corresponds approximately to the normal population. […] There is no evidence of effective interventions for prevention and early detection. […] Patients with HCL have an increased risk of infections at initial manifestation and after therapy with purine analogs. 50-60% of patients develop neutropenia after cladribine therapy. Myelosuppression is less common after pentostatin therapy. The risk of therapy-associated infections is approximately 20-30%.

• #32

  https://link.springer.com/article/10.1007/s00277-020-04349-z

  A high incidence of cutaneous adverse drug reactions, especially in treated patients with HCL, was observed, particularly in patients receiving cladribine and concomitant medications. […] Finally, the use of antileukemic drugs in HCL predisposes the patient to viral, fungal, and bacterial skin infections, including opportunistic infections.

• #33 SEER Hematopoietic and Lymphoid Neoplasm Database

  https://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5399/

  Epidemiology and Mortality: Age: 50 years median age Incidence: 2% of lymphoid leukemias Sex: male predominance Survival: 10 years survival rate 90%

• #34 Hairy cell leukemia: Outlook, treatment, and what to expect

  https://www.medicalnewstoday.com/articles/hairy-cell-leukemia-prognosis

  Treatment with cladribine has led to roughly 85% complete remission and around 10% partial response in people with HCL. […] The overall outlook for people with HCL is good. Treatment with chemotherapy drugs, such as cladribine and pentostatin, is highly effective and may result in long-term remission.

• #35 Hairy cell leukemia and COVID-19 adaptation of treatment guidelines | Leukemia

  https://www.nature.com/articles/s41375-021-01257-7

  Standard treatment options in classic HCL (cHCL) result in high response rates and near normal life expectancy. […] However, the disease itself and the recommended standard treatment are associated with profound and prolonged immunosuppression, increasing susceptibility to infections and the risk for a severe course of COVID-19. […] The Hairy Cell Leukemia Foundation (HCLF) has recently convened experts and discussed different clinical strategies for the management of these patients. […] The new recommendations adapt the 2017 consensus for the diagnosis and management with cHCL to the current COVID-19 pandemic. […] They underline the option of active surveillance in patients with low but stable blood counts, consider the use of targeted and non-immunosuppressive agents as first-line treatment for cHCL, and give recommendations on preventive measures against COVID-19.

• #36 Hairy cell leukemia: Outlook, treatment, and what to expect

  https://www.medicalnewstoday.com/articles/hairy-cell-leukemia-prognosis

  Hairy cell leukemia is a rare type of blood cancer that can affect adults. […] According to the National Organization for Rare Disorders, HCL is more common in males over the age of 50 years. […] HCL affects roughly 6,000 people in the United States, with around 600800 new diagnoses each year. Around 12% of all adult leukemias are HCL. […] According to a 2020 study, people with HCL have an increased risk of secondary cancer. […] People with HCL must attend regular cancer screenings to detect any early signs of secondary cancer. […] The study found that the 10-year survival rate was worse in African American participants than in those of other racial groups. […] The researchers concluded that the biological, socioeconomic, and health system factors contributing to this disparity need further investigation.

• #37 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia-guidelines.info/en/onkopedia/guidelines/hairy-cell-leukemia

  HCL is rare with an incidence of about 0.3-0.4/100,000 persons (crude disease rate). In Germany, approximately 70 women and 220 men develop the disease annually. Incidence and distribution are concordant with data from other countries. The disease accounts for about 3% of lymphocytic leukemias in the current evaluation. The median age of onset in Germany was most recently between 62 (men) and 65 (women) years. First diagnoses before the age of 30 are very rare, men are affected significantly more often than women in all age groups. […] In the last decade, a trend towards a somewhat earlier age of onset of disease has been seen in the centers. This is presumably a consequence of the greater use of general screening measures by younger people, with performance of a blood count. As a result, cytopenias are diagnosed earlier, i.e. before the onset of clinical symptoms.

• #38 Introduction and Epidemiology of Hairy Cell Leukemia

  https://www.onclive.com/view/introduction-and-epidemiology-of-hairy-cell-leukemia

  I also wanted to mention that the situation of massive splenomegaly and significant constitutional symptoms is becoming less common, mainly because people are getting routine blood screening. Frequently patients are found to be slightly cytopenic on a routine blood evaluation, and then further work-up shows hairy cell leukemia. […] It’s fortunate that we are catching these patients a lot earlier, and were not having the massive splenomegaly and splenic ruptures that used to be seen.

• #39 Hairy cell leukemia and COVID-19 adaptation of treatment guidelines | Leukemia

  https://www.nature.com/articles/s41375-021-01257-7

  At present, there are no updated published data on the infection rates with SARS-CoV-2 in HCL patients. […] Experiences with the course of COVID-19 in HCL patients are limited to case reports, and personal communications. […] Despite this lack of evidence, HCL patients and their treating physicians ask for guidance. […] Recommendations are based on the 2017 consensus for the diagnosis and management with cHCL, and on the increasing evidence on the adverse course of COVID-19 in patients with hematological malignancies. […] Consequently, some recommendations of the published guidelines require adaptation. […] The serious nature of these infections results both from the intrinsic immunosuppression associated with this leukemia, and its treatment. […] Several reports have highlighted the increased risk of poor outcome following COVID-19 infection in patients with a hematologic malignancy, but the comparative experience in cHCL has not been specifically well-documented.

• #40 Hairy Cell Leukemia Research — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/published-research

  During the COVID-19 Pandemic, 253 of 503 patients with hairy cell leukemia (HCL) or the high-risk variant HCLv had evidence of COVID-19. […] Standard treatment options in classic HCL (cHCL) result in high response rates and near normal life expectancy. […] The development of consensus guidelines for this disease offers a framework for continued enhancement of the outcome for patients. […] A significant body of literature has been generated related to the detection of measurable residual disease (MRD) at the time of achieving complete remission (CR) in patients with hairy cell leukemia (HCL).

Zobacz więcej