Epidemiology of Hairy Cell Leukemia (HCL) and HCL-Like Disorders
Main Article Content
Abstract
Hairy cell leukemia (HCL) is a very rare and well-defined entity that is characterized by the presence of hairy cells expressing the characteristic markers CD11c, CD25, CD103 and CD123 and in most cases by the presence of the BRAFV600E mutation. The incidence rate, directly adjusted to the 2000 United States standard population is estimated at 0.62 per 100 000 person-years (PA) in white men, 0.21 in black men, 0.20 in Asian men and 0.06 American Indian or Alaska native men. Based on the estimated 2019 leukemia incidence, of 61,780 in the United States, approximately 1,240 new HCL cases are expected per year, with only 60–75 new patients having a variant form of HCL each year. In France, the median age of patients at diagnosis is 63 years in men and 59 years in women. There is a strong male predominance, with a sex ratio of 5:1. HCL is a malignant disorder with a good prognosis, with a standardized average survival at 1 year and 5 years of 95% (95% CI: 91-97 for both). The etiology of HCL remains unknown. The risk of secondary cancers is high, especially that of another malignant hematologic disorder. This high risk justifies the need for prolonged hematological monitoring.
HCL must be distinguished from other HCL-like disorders, including the variant form of HCL (HCL-V) or splenic diffuse red pulp lymphoma (SDRPL), warranting the development of international epidemiologic studies.
Keywords:
Hairy cell leukemia, epidemiology, incidence, survival, second malignancies
Article Details
How to Cite
TROUSSARD, Xavier.
Epidemiology of Hairy Cell Leukemia (HCL) and HCL-Like Disorders.
Medical Research Archives, [S.l.], v. 8, n. 10, oct. 2020.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/2259>. Date accessed: 21 dec. 2024.
doi: https://doi.org/10.18103/mra.v8i10.2259.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
1. Matutes E, Morilla R, Owusu-Ankomah et al. The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B-cell disorders with hairy or villous lymphocytes. Leuk Lymphoma. 1994;14(Suppl1):57-61.
2. Tiacci E, Trifonov V, Schiavoni G et al. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011 Jun 16; 364(24):2305-15.
3. Maitre E, Cornet E, Troussard X. Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment. Am J Hematol. 2019 Dec; 94(12):1413-1422.
4. Paillassa J, Cornet E, Noel S, et al. Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up. Blood Cancer J. 2020;10(5):62.
5. Robak T. Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011;37(1):3-10.
6. Matutes E, Martínez-Trillos A, Campo E. Hairy cell leukaemia-variant: Disease features and treatment. Best Pract Res Clin Haematol. 2015;28(4):253-263.
7. Traverse-Glehen A, Baseggio L, Bauchu EC, et al. Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?. Blood. 2008;111(4):2253-2260.
8. Traverse-Glehen A, Baseggio L, Salles G, Coiffier B, Felman P, Berger F. Splenic diffuse red pulp small-B cell lymphoma: toward the emergence of a new lymphoma entity. Discov Med. 2012;13(71):253-265.
9. Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001.Blood, 2006,107, 265-276.
10. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2019. CA Cancer J Clin. 2019;69(1):7-34.
11. Gatta G, Capocaccia R, Botta L et al. RARECAREnet working group. Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol. 2017 Aug; 18(8):1022-1039.
12. Troussard X, E. Cornet, Monnereau A, Le Guyader‑Peyrou S. Leucémie à tricholeucocytes dans Estimations nationales de l’incidence et de la mortalité par cancer en France métropolitaine. Volume 2– Hémopathies malignes. Le Guyader-Peyrou S, Defossez G, Dantony E.
13. Robak T. Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011;37(1):3-10.
14. Matutes E, Wotherspoon A, Catovsky D. The variant form of hairy-cell leukaemia. Best Pract Res Clin Haematol 2003;16: 41–56.
15. Durham BH, Getta B, Dietrich S, et al. Genomic analysis of hairy cell leukemia identifies novel recurrent genetic alterations. Blood. 2017;130(14):1644-1648.
16. Curiel-Olmo S, Mondéjar R, Almaraz C, et al. Splenic diffuse red pulp small B-cell lymphoma displays increased expression of cyclin D3 and recurrent CCND3 mutations. Blood. 2017;129(8):1042-1045.
17. Waterfall JJ, Arons E, Walker RL, et al. High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias. Nat Genet. 2014;46(1):8-10.
18. Favre R, Manzoni D, Traverse-Glehen A, et al. Usefulness of CD200 in the differential diagnosis of SDRPL, SMZL, and HCL. Int J Lab Hematol. 2018;40(4):e59-e62.
19. Baseggio L, Traverse-Glehen A, Callet-Bauchu E, et al. Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL). Hematol Oncol. 2011;29(1):47-51.
20. Pulte D, Weberpals J, Jansen L et al. Survival for patients with rare haematologic malignancies: Changes in the early 21st century. Eur J Cancer. 2017 Oct; 84:81-87.
21. Chandran R, Gardiner SK, Smith SD, Spurgeon SE. Improved survival in hairy cell leukaemia over three decades: a SEER database analysis of prognostic factors. Br J Haematol. 2013 Nov; 163(3):407-9.
22. Giri S, Shrestha R, Pathak R, Bhatt VR. Racial Differences in the Overall Survival of Hairy Cell Leukemia in the United States: A Population-Based Analysis of the Surveillance, Epidemiology, and End Results Database. Clin Lymphoma Myeloma Leuk. 2015 Aug; 15(8):484-8.
23. Madanat YF, Rybicki L, Radivoyevitch T, et al. Long-Term Outcomes of Hairy Cell Leukemia treated with Purine Analogs: A Comparison With the General Population. Clin Lymphoma Myeloma Leuk. 2017;17(12):857-862.
24. Dinmohamed AG, Posthuma EFM, Visser O, Kater AP, Raymakers RAP, Doorduijn JK. Relative survival reaches a plateau in hairy cell leukemia: a population-based analysis in The Netherlands. Blood. 2018 Mar 22; 131(12):1380-1383.
25. Pemov A, Pathak A, Jones SJ et al. In search of genetic factors predisposing to familial hairy cell leukemia (HCL): exome-sequencing of four multiplex HCL pedigrees. Leukemia. 2020 Jan 28. doi: 10.1038/s41375-019-0702-7.
26. Goldin LR, Björkholm M, Kristinsson SY,Turesson I Landgren O. Elevated risk of chronic lymphocytic leukemia and other indolent non-Hodgkin’s lymphomas among relatives of patients with chronic lymphocytic leukemia. Hematologica 2009; 94(5), 647-653.
27. Sud A, Chattopadhyay S, Thomsen H et al. Analysis of 153 115 patients with hematological malignancies refines the spectrum of familial risk. Blood. 2019 Sep 19; 134(12):960-969.
28. Orsi L, Delabre L, Monnereau A et al. Occupational exposure to pesticides and lymphoid neoplasms among men: results of a French case-control study. Occup Environ Med. 2009 May; 66(5):291-8.
29. Clavel J, Mandereau L, Cordier S, Le Goaster C, Hémon D, Conso F, Flandrin G. Hairy cell leukaemia, occupation, and smoking. Br J Haematol. 1995 Sep; 91(1):154-61.
30. Tadmor T, Polliack A. Epidemiology and environmental risk in hairy cell leukemia. Best Pract Res Clin Haematol. 2015 Dec; 28(4):175-9.
31. Monnereau A, Slager SL, Hughes AM et al. Medical history, lifestyle, and occupational risk factors for hairy cell leukemia: the InterLymph Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014 Aug; 2014(48):115-24.
32. Troussard X, Henry-Amar M, Flandrin G. Second cancer risk after interferon therapy?. Blood. 1994;84(9):3242-3244.
33. Federico M, Zinzani PL, Frassoldati A, et al. Risk of second cancer in patients with hairy cell leukemia: long-term follow-up. J Clin Oncol. 2002;20(3):638-646.
34. Hisada M, Chen BE, Jaffe ES, Travis LB. Second cancer incidence and cause-specific mortality among 3104 patients with hairy cell leukemia: a population-based study. J Natl Cancer Inst. 2007;99(3):215-222.
35. da Silva WF, Neto AC, da Rosa LI, et al. Outcomes and second neoplasms in hairy cell leukemia: A retrospective cohort. Leuk Res. 2019;83:106165.
36. Cornet E, Tomowiak C, Tanguy-Schmidt A et al. Long-term follow-up and second malignancies in 487 patients with hairy cell leukaemia. Br J Haematol. 2014 Aug; 166(3):390-400.
37. Au WY, Klasa RJ, Gallagher R, Le N, Gascoyne RD, Connors JM. Second malignancies in patients with hairy cell leukemia in britishcolumbia: a 20-year experience. Blood. 1998;92(4):1160-1164.
38. Zheng G, Chattopadhyay S, Sud A et al. Types of second primary cancers influence survival in chronic lymphocytic and hairy cell leukemia patients. Blood Cancer J. 2019 Mar 26; 9(4):40.
2. Tiacci E, Trifonov V, Schiavoni G et al. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011 Jun 16; 364(24):2305-15.
3. Maitre E, Cornet E, Troussard X. Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment. Am J Hematol. 2019 Dec; 94(12):1413-1422.
4. Paillassa J, Cornet E, Noel S, et al. Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up. Blood Cancer J. 2020;10(5):62.
5. Robak T. Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011;37(1):3-10.
6. Matutes E, Martínez-Trillos A, Campo E. Hairy cell leukaemia-variant: Disease features and treatment. Best Pract Res Clin Haematol. 2015;28(4):253-263.
7. Traverse-Glehen A, Baseggio L, Bauchu EC, et al. Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?. Blood. 2008;111(4):2253-2260.
8. Traverse-Glehen A, Baseggio L, Salles G, Coiffier B, Felman P, Berger F. Splenic diffuse red pulp small-B cell lymphoma: toward the emergence of a new lymphoma entity. Discov Med. 2012;13(71):253-265.
9. Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001.Blood, 2006,107, 265-276.
10. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2019. CA Cancer J Clin. 2019;69(1):7-34.
11. Gatta G, Capocaccia R, Botta L et al. RARECAREnet working group. Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol. 2017 Aug; 18(8):1022-1039.
12. Troussard X, E. Cornet, Monnereau A, Le Guyader‑Peyrou S. Leucémie à tricholeucocytes dans Estimations nationales de l’incidence et de la mortalité par cancer en France métropolitaine. Volume 2– Hémopathies malignes. Le Guyader-Peyrou S, Defossez G, Dantony E.
13. Robak T. Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011;37(1):3-10.
14. Matutes E, Wotherspoon A, Catovsky D. The variant form of hairy-cell leukaemia. Best Pract Res Clin Haematol 2003;16: 41–56.
15. Durham BH, Getta B, Dietrich S, et al. Genomic analysis of hairy cell leukemia identifies novel recurrent genetic alterations. Blood. 2017;130(14):1644-1648.
16. Curiel-Olmo S, Mondéjar R, Almaraz C, et al. Splenic diffuse red pulp small B-cell lymphoma displays increased expression of cyclin D3 and recurrent CCND3 mutations. Blood. 2017;129(8):1042-1045.
17. Waterfall JJ, Arons E, Walker RL, et al. High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias. Nat Genet. 2014;46(1):8-10.
18. Favre R, Manzoni D, Traverse-Glehen A, et al. Usefulness of CD200 in the differential diagnosis of SDRPL, SMZL, and HCL. Int J Lab Hematol. 2018;40(4):e59-e62.
19. Baseggio L, Traverse-Glehen A, Callet-Bauchu E, et al. Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL). Hematol Oncol. 2011;29(1):47-51.
20. Pulte D, Weberpals J, Jansen L et al. Survival for patients with rare haematologic malignancies: Changes in the early 21st century. Eur J Cancer. 2017 Oct; 84:81-87.
21. Chandran R, Gardiner SK, Smith SD, Spurgeon SE. Improved survival in hairy cell leukaemia over three decades: a SEER database analysis of prognostic factors. Br J Haematol. 2013 Nov; 163(3):407-9.
22. Giri S, Shrestha R, Pathak R, Bhatt VR. Racial Differences in the Overall Survival of Hairy Cell Leukemia in the United States: A Population-Based Analysis of the Surveillance, Epidemiology, and End Results Database. Clin Lymphoma Myeloma Leuk. 2015 Aug; 15(8):484-8.
23. Madanat YF, Rybicki L, Radivoyevitch T, et al. Long-Term Outcomes of Hairy Cell Leukemia treated with Purine Analogs: A Comparison With the General Population. Clin Lymphoma Myeloma Leuk. 2017;17(12):857-862.
24. Dinmohamed AG, Posthuma EFM, Visser O, Kater AP, Raymakers RAP, Doorduijn JK. Relative survival reaches a plateau in hairy cell leukemia: a population-based analysis in The Netherlands. Blood. 2018 Mar 22; 131(12):1380-1383.
25. Pemov A, Pathak A, Jones SJ et al. In search of genetic factors predisposing to familial hairy cell leukemia (HCL): exome-sequencing of four multiplex HCL pedigrees. Leukemia. 2020 Jan 28. doi: 10.1038/s41375-019-0702-7.
26. Goldin LR, Björkholm M, Kristinsson SY,Turesson I Landgren O. Elevated risk of chronic lymphocytic leukemia and other indolent non-Hodgkin’s lymphomas among relatives of patients with chronic lymphocytic leukemia. Hematologica 2009; 94(5), 647-653.
27. Sud A, Chattopadhyay S, Thomsen H et al. Analysis of 153 115 patients with hematological malignancies refines the spectrum of familial risk. Blood. 2019 Sep 19; 134(12):960-969.
28. Orsi L, Delabre L, Monnereau A et al. Occupational exposure to pesticides and lymphoid neoplasms among men: results of a French case-control study. Occup Environ Med. 2009 May; 66(5):291-8.
29. Clavel J, Mandereau L, Cordier S, Le Goaster C, Hémon D, Conso F, Flandrin G. Hairy cell leukaemia, occupation, and smoking. Br J Haematol. 1995 Sep; 91(1):154-61.
30. Tadmor T, Polliack A. Epidemiology and environmental risk in hairy cell leukemia. Best Pract Res Clin Haematol. 2015 Dec; 28(4):175-9.
31. Monnereau A, Slager SL, Hughes AM et al. Medical history, lifestyle, and occupational risk factors for hairy cell leukemia: the InterLymph Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014 Aug; 2014(48):115-24.
32. Troussard X, Henry-Amar M, Flandrin G. Second cancer risk after interferon therapy?. Blood. 1994;84(9):3242-3244.
33. Federico M, Zinzani PL, Frassoldati A, et al. Risk of second cancer in patients with hairy cell leukemia: long-term follow-up. J Clin Oncol. 2002;20(3):638-646.
34. Hisada M, Chen BE, Jaffe ES, Travis LB. Second cancer incidence and cause-specific mortality among 3104 patients with hairy cell leukemia: a population-based study. J Natl Cancer Inst. 2007;99(3):215-222.
35. da Silva WF, Neto AC, da Rosa LI, et al. Outcomes and second neoplasms in hairy cell leukemia: A retrospective cohort. Leuk Res. 2019;83:106165.
36. Cornet E, Tomowiak C, Tanguy-Schmidt A et al. Long-term follow-up and second malignancies in 487 patients with hairy cell leukaemia. Br J Haematol. 2014 Aug; 166(3):390-400.
37. Au WY, Klasa RJ, Gallagher R, Le N, Gascoyne RD, Connors JM. Second malignancies in patients with hairy cell leukemia in britishcolumbia: a 20-year experience. Blood. 1998;92(4):1160-1164.
38. Zheng G, Chattopadhyay S, Sud A et al. Types of second primary cancers influence survival in chronic lymphocytic and hairy cell leukemia patients. Blood Cancer J. 2019 Mar 26; 9(4):40.