Skip to main content

Advertisement

SpringerLink
Log in

Biology and Treatment of Hairy Cell Leukemia

  • Leukemia (PH Wiernik, Section Editor)
  • Published:
Current Treatment Options in Oncology Aims and scope Submit manuscript

Opinion statement

Despite its rarity, hairy cell leukemia (HCL) remains a fascinating disease and the physiopathology is becoming more and more understood. The accurate diagnosis of HCL relies on the recognition of hairy cells by morphology and flow cytometry (FCM) in the blood and/or bone marrow (BM). The BRAF V600E mutation, an HCL-defining mutation, represents a novel diagnostic parameter and a potential therapeutic target. The precise cellular origin of HCL is a late-activated postgerminal center memory B cell. BRAF mutations were detected in hematopoietic stem cells (HSCs) of patients with HCL, suggesting that this is an early HCL-defining event. Watch-and-wait strategy is necessary in approximately 10% of asymptomatic HCL patients, sometimes for several years. Purine analogs (PNAs) are the established first-line options for symptomatic HCL patients. In second-line treatment, chemoimmunotherapy combining PNA plus rituximab should be considered in high-risk HCL patients. The three options for relapsed/refractory HCL patients include recombinant immunoconjugates targeting CD22, BRAF inhibitors, and BCR inhibitors. The clinical interest to investigate blood minimal residual disease (MRD) was recently demonstrated, with a high risk of relapse in patients with positive testing for MRD and a low risk in patients with negative testing. However, efforts must be made to standardize MRD analyses in the near future. Patients with HCL are at risk of second malignancies. The increased risk could be related to the disease and/or the treatment, and the respective role of PNAs in the development of secondary malignancies remains a topic of debate.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the world health organization classification of lymphoid neoplasms. Blood. 2016;127:2375–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Kreitman RJ. Hairy cell leukemia: present and future directions. Leuk Lymphoma. 2019;60:2869–79.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Paltiel O, Adler B, Barchana M, et al. A population-based study of hairy cell leukemia in Israel. Eur J Haematol. 2006;77:372–7.

    Article  PubMed  Google Scholar 

  4. Dinmohamed AG, Posthuma EFM, Visser O, et al. Relative survival reaches a plateau in hairy cell leukemia: a population-based analysis in The Netherlands. Blood. 2018;131:1380–3.

    Article  CAS  PubMed  Google Scholar 

  5. Morton LM, Wang SS, Devesa SS, et al. Lymphoma incidence patterns by WHO subtype in the United States, 1992–2001. Blood. 2006;107:265–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Giri S, Shrestha R, Pathak R, et al. 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;15:484–8.

    Article  PubMed  Google Scholar 

  7. Le Guyader-Peyrou S, Defossez G, Dantony E, et al.. Estimations nationales de l’incidence et de la mortalité par cancer en France métropolitaine entre 1990 et 2018. Hémopathies Malignes, Étude Partir Regist Cancers Réseau Fr. 2019; 2:169.

  8. Pemov A, Pathak A, Jones SJ, Dewan R, Merberg J, Karra S, et al. In search of genetic factors predisposing to familial hairy cell leukemia (HCL): exome-sequencing of four multiplex HCL pedigrees. Leukemia. 2020.

  9. Villemagne B, Bay J-O, Tournilhac O, Chaleteix C, Travade P. Two new cases of familial hairy cell leukemia associated with HLA haplotypes A2, B7, Bw4, Bw6. Leuk Lymphoma. 2005;46:243–5.

    Article  CAS  PubMed  Google Scholar 

  10. 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. Haematologica. 2009;94:647–53.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Robak T. Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011;37:3–10.

    Article  PubMed  Google Scholar 

  12. 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;111:2253–60.

    Article  CAS  PubMed  Google Scholar 

  13. Jallades L, Baseggio L, Sujobert P, et al. Exome sequencing identifies recurrent BCOR alterations and the absence of KLF2, TNFAIP3 and MYD88 mutations in splenic diffuse red pulp small B-cell lymphoma. Haematologica. 2017;102:1758–66.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Durot E, Quinquenel A, Kleiber JC, et al. Skeletal involvement of hairy cell leukemia. Clin Case Rep. 2018;6:1651–2.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Notarfranchi L, Russo F, Re F, et al. Hairy cell leukaemia mimicking multiple myeloma. Lancet Oncol. 2019;20:e187.

    Article  PubMed  Google Scholar 

  16. Zhang X, Machii T, Matsumura I, et al. Constitutively activated Rho guanosine triphosphatases regulate the growth and morphology of hairy cell leukemia cells. Int J Hematol. 2003;77:263–73.

    Article  CAS  PubMed  Google Scholar 

  17. Salem DA, Scott D, McCoy CS, et al. Differential expression of CD43, CD81, and CD200 in classic versus variant hairy cell leukemia. Cytometry B Clin Cytom. 2019;96:275–82.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. • Matutes E, Morilla R, Owusu-Ankomah K, 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(Suppl 1):57–61 After analyzing the immunological profile of abnormal lymphoid cells from 194 patients with a B cell chronic lymphoproliferative disorder associated with circulating hairy or villous lymphocytes, a scoring system was proposed considering the reactivity with each of the four markers (CD11c, CD103, CD123 and CD25) and gives 1 point if positive and 0 points if negative. Scores range from 4 (typical of HCL) to 0 (atypical of HCL). 98% of HCL had high scores (3 or 4) whereas 88% of HCL-V and 77% of SLVL scored 1 or 2 and no single case scored 3 or 4.

    PubMed  Google Scholar 

  19. Poret N, Fu Q, Guihard S, et al. CD38 in hairy cell leukemia is a marker of poor prognosis and a new target for therapy. Cancer Res. 2015;75:3902–11.

    Article  CAS  PubMed  Google Scholar 

  20. •• Grever MR, Abdel-Wahab O, Andritsos LA, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood. 2017;129:553–60 Hairy Cell Leukemia Foundation convened an international conference with a large panel of experts to provide common definitions and structure to guide current management. The development of consensus guidelines for this disease offers a framework for continued enhancement of the outcome for Hairy Cell Leukemia patients.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. 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:e59–62.

    Article  CAS  PubMed  Google Scholar 

  22. 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;29:47–51.

    Article  CAS  PubMed  Google Scholar 

  23. •• Tiacci E, Trifonov V, Schiavoni G, et al. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011;364:2305–15 The BRAF V600E mutation was identified by whole-exome sequencing (WES) then confirmed by Sanger sequencing in 47 HCL patients. Conversely, none of the 195 patients with other B cell chronic lymphoproliferative disorders who were evaluated carried the BRAF V600E variant, including 38 patients with splenic marginal-zone lymphomas or unclassifiable splenic lymphomas or leukemias.

  24. • Tschernitz S, Flossbach L, Bonengel M, et al. Alternative BRAF mutations in BRAF V600E-negative hairy cell leukaemias. Br J Haematol. 2014;165:529–33 In cases of BRAFV600E negative hairy cell leukemia, the presence of alternative BRAF mutations must be sought, particularly in exon 11 of BRAF.

    Article  CAS  PubMed  Google Scholar 

  25. •• 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;46:8–10 Hairy Cell Leukemia variant and IGHV 4-34 Hairy Cell Leukemia cases can be characterized by a high prevalence of MAP2K1 mutations and the absence of BRAF V600E mutaions.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  26. Mason EF, Brown RD, Szeto DP, et al. Detection of activating MAP2K1 mutations in atypical hairy cell leukemia and hairy cell leukemia variant. Leuk Lymphoma. 2017;58:233–6.

    Article  PubMed  Google Scholar 

  27. •• Maitre E, Bertrand P, Maingonnat C, et al. New generation sequencing of targeted genes in the classical and the variant form of hairy cell leukemia highlights mutations in epigenetic regulation genes. Oncotarget. 2018;9:28866–76 When selecting a panel of 21 relevant genes, BRAFV600E was found in 90% of Hairy Cell Leukemia patients and was associated with other mutations in 33% of cases. All patients with hairy Cell Leukemia Variant had mutations in epigenetic regulatory genes: KDM6A, CREBBP or ARID1A. The analysis of sequential samples (at diagnosis and relapse) showed the presence of new subclonal mutations and variations of the mutated allele frequency. The analysis opens new perspectives for personalized medicine for patients with Hairy Cell Leukemia and Hairy Cell Leukemia variant.

  28. Dietrich S, Hullein J, Lee SC, et al. Recurrent CDKN1B (p27) mutations in hairy cell leukemia. Blood. 2015;126:1005–8.

    Article  CAS  PubMed  Google Scholar 

  29. •• Forconi F, Sozzi E, Cencini E, et al. Hairy cell leukemias with unmutated IGHV genes define the minor subset refractory to single-agent cladribine and with more aggressive behavior. Blood. 2009;114:4696–702 In 58 patients with Hairy Cell Leukemia, the IGHV profile was unmutated in 6 patients and mutated in 52 cases. Beneficial responses were obtained with purine nucleoside analogs in 91%, whereas treatment failures were observed in 9% of cases. Failures were associated significantly with an unmutated IGHV profile, leukocytosis, and bulky spleen. The unmutated HCL not benefiting from cladribine characteristically had bulky spleen, leukocytosis and TP53 defects, and progressed rapidly after first treatment. The data suggest that unmuttaed HC identify a minor subgroup failing cladribine treatment and with more aggressive disease.

  30. Durham BH, Getta B, Dietrich S, et al. Genomic analysis of hairy cell leukemia identifies novel recurrent genetic alterations. Blood. 2017;130:1644–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Hockley SL, Else M, Morilla A, et al. The prognostic impact of clinical and molecular features in hairy cell leukaemia variant and splenic marginal zone lymphoma. Br J Haematol. 2012;158:347–54.

    Article  CAS  PubMed  Google Scholar 

  32. Konig EA, Kusser WC, Day C, et al. p53 mutations in hairy cell leukemia. Leukemia. 2000;14:706–11.

    Article  CAS  PubMed  Google Scholar 

  33. Cornet E, Delmer A, Feugier P, et al. Recommendations of the SFH (French society of haematology) for the diagnosis, treatment and follow-up of hairy cell leukaemia. Ann Hematol. 2014;93:1977–83.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Xi L, Arons E, Navarro W, et al. Both variant and IGHV4-34-expressing hairy cell leukemia lack the BRAF V600E mutation. Blood. 2012;119:3330–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Curiel-Olmo S, Mondejar 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;129:1042–5.

    Article  CAS  PubMed  Google Scholar 

  36. Basso K, Liso A, Tiacci E, et al. Gene expression profiling of hairy cell leukemia reveals a phenotype related to memory B cells with altered expression of chemokine and adhesion receptors. J Exp Med. 2004;199:59–68.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. •• Arribas AJ, Rinaldi A, Chiodin G, et al. Genome-wide promoter methylation of hairy cell leukemia. Blood Adv. 2019;3:384–96 Hairy Cell Leukemia has a methylation signature distinct from each B cell chronic lymphoproliferative disorder, including the closest entity, Splenic Marginal Zone Lymphoma. Comparison with normal B cell subsets revealed the strongest similarity with postgerminal center (GC) B cells and a clear separation from pre-GC and GC cellular programs.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. •• Chung SS, Kim E, Park JH, et al. Hematopoietic stem cell origin of BRAFV600E mutations in hairy cell leukemia. Sci Transl Med. 2014;6:238ra271 The study shows the presence of BRAFV600E mutation in hairy cells but also in hematopoietic stem cells of patients with Hairy Cell Leukemia. In addition, expression of BRAFV600E in murine hematopoietic/progenitor cells can cause HCL-like disease. The data demonstrate that the mature B cell malignancies can initiate in the hematopoietic stem cell compartment.

    Google Scholar 

  39. Falini B, Martelli MP, Tiacci E. BRAF V600E mutation in hairy cell leukemia: from bench to bedside. Blood. 2016;128:1918–27.

    Article  CAS  PubMed  Google Scholar 

  40. Tiacci E, Liso A, Piris M, et al. Evolving concepts in the pathogenesis of hairy-cell leukaemia. Nat Rev. Cancer. 2006;6:437–48.

    Article  CAS  PubMed  Google Scholar 

  41. Sivina M, Burger JA. The importance of the tissue microenvironment in hairy cell leukemia. Best Pract Res Clin Haematol. 2015;28:208–16.

    Article  PubMed  Google Scholar 

  42. Sivina M, Kreitman RJ, Arons E, et al. The bruton tyrosine kinase inhibitor ibrutinib (PCI-32765) blocks hairy cell leukaemia survival, proliferation and B cell receptor signalling: a new therapeutic approach. Br J Haematol. 2014;166:177–88.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Pettirossi V, Santi A, Imperi E, et al. BRAF inhibitors reverse the unique molecular signature and phenotype of hairy cell leukemia and exert potent antileukemic activity. Blood. 2015;125:1207–16.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Garnache Ottou F, Chandesris MO, Lhermitte L, et al. Peripheral blood 8 colour flow cytometry monitoring of hairy cell leukaemia allows detection of high-risk patients. Br J Haematol. 2014;166:50–9.

    Article  CAS  PubMed  Google Scholar 

  45. Brown NA, Betz BL, Weigelin HC, et al. Evaluation of allele-specific PCR and immunohistochemistry for the detection of BRAF V600E mutations in hairy cell leukemia. Am J Clin Pathol. 2015;143:89–99.

    Article  PubMed  Google Scholar 

  46. Gupta GK, Sun X, Yuan CM, et al. Usefulness of dual immunohistochemistry staining in detection of hairy cell leukemia in bone marrow. Am J Clin Pathol. 2019. https://doi.org/10.1093/ajcp/aqz171.

  47. Chandran R, Gardiner SK, Smith SD, et al. Improved survival in hairy cell leukaemia over three decades: a SEER database analysis of prognostic factors. Br J Haematol. 2013;163:407–9.

    Article  PubMed  Google Scholar 

  48. • Chihara D, Kantarjian H, O’Brien S, et al. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016;174:760–6 The study demonstrates the interest of chemoimmunotherapy in untreated and relapsed patients with Hairy Cell Leukemia. Complete response were achieved in 100% of cases and minimal residual disease was undetectable in 94% of cases after treatment combining cladribine then rituximab one month later.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Chihara D, Arons E, Stetler-Stevenson M, et al. Randomized phase II study of cladribine with simultaneous or delayed rituximab in patients with untreated hairy cell leukemia. J Clin Oncol. 2019;37:7003.

    Article  Google Scholar 

  50. Burotto M, Stetler-Stevenson M, Arons E, et al. Bendamustine and rituximab in relapsed and refractory hairy cell leukemia. Clin Cancer Res. 2013;19:6313–21.

    Article  CAS  PubMed  Google Scholar 

  51. Visentin A, Imbergamo S, Frezzato F, et al. Bendamustine plus rituximab is an effective first-line treatment in hairy cell leukemia variant: a report of three cases. Oncotarget. 2017;8:110727–110,731.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Kreitman RJ, Tallman MS, Robak T, et al. Phase I trial of anti-CD22 recombinant immunotoxin moxetumomab pasudotox (CAT-8015 or HA22) in patients with hairy cell leukemia. J Clin Oncol. 2012;30:1822–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. •• Kreitman RJ, Dearden C, Zinzani PL, et al. Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia. Leukemia. 2018;32:1768–77 Moxetumomab pasudotox, a recombinant CD22-targeting immunotoxin was evaluated in 80 patients with relapsed/refractory Hairy Cell Leukemia. The durable complete response (CR) rate was 30%, CR rate was 41%, and objective response rate was 75%; 80% of patients achieved hematologic remission. Among complete responders, 85% achieved MRD negativity. The treatment is likely to induce a high rate of durable response and MRD eradication in heavily pretreated patients with HCL, with acceptable tolerability.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. •• Tiacci E, Park JH, De Carolis L, et al. Targeting mutant BRAF in relapsed or refractory hairy-cell leukemia. N Engl J Med. 2015;373:1733–47 The two phase-2 clinical trials indicate the effectiveness of high dose vemurafenif, a BRAf inhibitor, in patients with relapsed/refractory hairy cell leukemia. The overall response rates were 96% after a median of 8 weeks in the Italian study and 100% after a median of 12 weeks in the U.S. study. The rates of complete response were 35% and 42%, respectively. In the Italian trial, after a median follow-up of 23 months, the median relapse-free survival was 19 months among patients with a complete response and 6 months among those with a partial response; the median treatment-free survival was 25 months and 18 months, respectively. In the U.S. trial, at 1 year, the progression-free survival rate was 73% and the overall survival rate was 91%.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Troussard X, Montané L, Tiab M, et al. Vemurafenib in advanced patients with hairy cell leukemia (HCL): results of the Acsé phase II trial. Blood. 2017;130:156.

    Article  CAS  Google Scholar 

  56. Callahan MK, Rampal R, Harding JJ, et al. Progression of RAS-mutant leukemia during RAF inhibitor treatment. N Engl J Med. 2012;367:2316–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Yaktapour N, Meiss F, Mastroianni J, et al. BRAF inhibitor-associated ERK activation drives development of chronic lymphocytic leukemia. J Clin Invest. 2014;124:5074–84.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. •• Dietrich S, Pircher A, Endris V, et al. BRAF inhibition in hairy cell leukemia with low-dose vemurafenib. Blood. 2016;127:2847–55 The authors analyzed the course of 21 HCL patients treated with vemurafenib, a BRAF inhibitor, with individual dosing and low dose regimens (240–1920 mg/d; median treatment duration, 90 days). Complete remission was achieved in 40% and median event-free survival was 17 months. Treatment with low dose BRAF inhibitors represents an effective and relatively well tolerated alternative treatment.

    Article  CAS  PubMed  Google Scholar 

  59. Bohn JP, Pircher A, Wanner D, et al. Low-dose vemurafenib in hairy cell leukemia patients with active infection. Am J Hematol. 2019;94:E180–2.

    Article  PubMed  PubMed Central  Google Scholar 

  60. Kreitman RJ, Moreau P, Hutchings M, et al. Treatment with combination of dabrafenib and trametinib in patients with recurrent/refractory BRAF V600E-mutated hairy cell leukemia (HCL). Blood. 2018;132:391.

    Article  Google Scholar 

  61. Tiacci E, De Carolis L, Simonetti E, et al. The braf inhibitor vemurafenib combined with rituximab produces a high rate of deep and durable remissions in relapsed or refractory hairy cell leukemia: updated results of a phase-2 trial: S104. HemaSphere. 2019;3:110–1.

    Article  Google Scholar 

  62. Jones JA, Andritsos L, Ravandi F, et al. Safety and efficacy of the Bruton tyrosine kinase inhibitor ibrutinib in patients with hairy cell leukemia: interim results of a phase 2 study. Haematologica. 2015;100:313.

    Google Scholar 

  63. Goodman GR, Burian C, Koziol JA, et al. Extended follow-up of patients with hairy cell leukemia after treatment with cladribine. J Clin Oncol. 2003;21:891–6.

    Article  PubMed  Google Scholar 

  64. Au WY, Klasa RJ, Gallagher R, et al. Second malignancies in patients with hairy cell leukemia in british columbia: a 20-year experience. Blood. 1998;92:1160–4.

    Article  CAS  PubMed  Google Scholar 

  65. Hisada M, Chen BE, Jaffe ES, et al. Second cancer incidence and cause-specific mortality among 3104 patients with hairy cell leukemia: a population-based study. J Natl Cancer Inst. 2007;99:215–22.

    Article  PubMed  Google Scholar 

  66. Kampmeier P, Spielberger R, Dickstein J, et al. Increased incidence of second neoplasms in patients treated with interferon alpha 2b for hairy cell leukemia: a clinicopathologic assessment. Blood. 1994;83:2931–8.

    Article  CAS  PubMed  Google Scholar 

  67. Pawson R, A’Hern R, Catovsky D. Second malignancy in hairy cell leukaemia: no evidence of increased incidence after treatment with interferon alpha. Leuk Lymphoma. 1996;22:103–6.

    Article  CAS  PubMed  Google Scholar 

  68. Watts JM, Kishtagari A, Hsu M, et al. Melanoma and non-melanoma skin cancers in hairy cell leukaemia: a Surveillance, Epidemiology and End Results population analysis and the 30-year experience at Memorial Sloan Kettering Cancer Center. Br J Haematol. 2015;171:84–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Rosenberg JD, Burian C, Waalen J, et al. Clinical characteristics and long-term outcome of young hairy cell leukemia patients treated with cladribine: a single-institution series. Blood. 2014;123:177–83.

    Article  CAS  PubMed  Google Scholar 

  70. • 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;166:390–400 Of the 487 HCL patients, 18% had a familial history of cancers, 8% presented with malignancies before HCL diagnosis and 10% developed second malignancies after HCL was diagnosed. An excess incidence of second malignancies was observed, with a standardized incidence ratio (SIR) of 1·86 (95% confidence interval (CI): 1·34–2·51), with no significant difference between PNAs. For second hematological malignancies alone, the SIR was markedly increased at 5·32 (95% CI: 2·90–8·92).

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Hematology, Academic Hospital of Angers, Angers, Pays de la Loire, France

    Jérôme Paillassa MD

  2. Laboratory of Hematology, Academic Hospital of Caen, Caen, Normandy, France

    Xavier Troussard MD

  3. Laboratoire d’Hématologie Biologique, CHU de Caen, Avenue de la Côte de Nacre, 14 033, Caen Cedex, France

    Xavier Troussard MD

Authors
  1. Jérôme Paillassa MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Xavier Troussard MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Xavier Troussard MD.

Ethics declarations

Conflict of Interest

Jerôme Paillassa declares that he has no conflict of interest. Xavier Troussard is a consultant for Innate Pharma.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical collection on Leukemia

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Paillassa, J., Troussard, X. Biology and Treatment of Hairy Cell Leukemia. Curr. Treat. Options in Oncol. 21, 44 (2020). https://doi.org/10.1007/s11864-020-00732-0

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11864-020-00732-0

Keywords

Search

Navigation