Skip to main content
SpringerLink
Log in

Retrospective Analysis of Hairy Cell Leukemia Patients Treated with Different Modalities as First Line: Real-Life Experience Over 20 years

  • Original Article
  • Published:
Indian Journal of Hematology and Blood Transfusion Aims and scope Submit manuscript

Abstract

We aimed to analyze the characteristics and response rates of different treatment modalities in hairy cell leukemia patients over 20 diagnosed as hairy cell leukemia (HCL). Clinical data, response rates and survival outcome of the patients who were diagnosed with HCL were retrospectively analyzed. Fifty-two patients with a median age of 50 (28–87) years were enrolled in the study. 38 patients (73%) were male and male to female ratio was 2.7. First line therapy was cladrabine in 36 patients (69.2%). The overall response rate was 97%. CR and PR rates were 86.1% and 11.1%, respectively. Interferon was used in 10(19.2%) patients who were diagnosed before 2000s years. CR and PR rates were 70% and 30%, respectively. Although the CR rates were lower in IFN group, this difference could not be reached statistically significance (p = 0.24). The median follow up was 48 months (12–252). The median OS was not reached and median PFS was 150 months (95% CI, 116–214). The OS at 36 and 48 months were 95.9% and 92.3%, respectively and the PFS at 36 and 48 months were 90.2% and 83.4%, respectively. After the introduction of purine analogues, the fate of the HCL patients have been changed. Cladrabin achieved very high response rates in both young and older patients, in our study. Although relapse still constitutes a problem, another single dose of cladrabine results in good response rates.

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

Similar content being viewed by others

References

  1. Golomb HM, Catovsky D, Golde DW (1978) Hairy cell leukemia: a clinical review based on 71 cases. Ann Intern Med 89:677–683

    Article  CAS  Google Scholar 

  2. Bernstein L, Newton P, Ross RK (1990) Epidemiology of hairy cell leukemia in Los Angeles County. Cancer Res 50(12):3605–3609

    CAS  PubMed  Google Scholar 

  3. Quest GR, Johnton JB (2015) Clinical features and diagnosis of hairy cell leukemia. Best Pract Res Clin Haematol 28(4):180–192

    Article  Google Scholar 

  4. Flandrin G, Sigaux F, Sebahoun G, Bouffette P (1984) Hairy cell leukemia: clinical presentation and follow-up of 211 patients. Semin Oncol 11(4 Suppl 2):458–471

    CAS  PubMed  Google Scholar 

  5. Jones G, Parry-Jones N, Wilkins B, Else M, Catovsky D (2012) British Committee for Standards in Haematology. Revised guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant*. Br J Haematol 156(2):186–195

    Article  CAS  Google Scholar 

  6. Falini B, Tiacci E, Liso A, Basso K, Sabattini E, Pacini R et al (2004) Simple diagnostic assay for hairy cell leukaemia by immunocytochemical detection of annexin A1 (ANXA1). Lancet 363(9424):1869–1870

    Article  CAS  Google Scholar 

  7. Grever MR, Lozanski G (2011) Modern strategies for hairy cell leukemia. J Clin Oncol 29(5):583–590

    Article  Google Scholar 

  8. Grever MR (2010) How I treat hairy cell leukemia. Blood 115(1):21–28

    Article  CAS  Google Scholar 

  9. Golomb HM, Vardiman JW (1983) Response to splenectomy in 65 patients with hairy cell leukemia: an evaluation of spleen weight and bone marrow involvement. Blood 61(2):349–352

    Article  CAS  Google Scholar 

  10. Quesada JR, Hersh EM, Manning J, Reuben J, Keating M, Schnipper E, Itri L, Gutterman JU (1986) Treatment of hairy cell leukemia with recombinant alpha-interferon. Blood 68(2):493–497

    Article  CAS  Google Scholar 

  11. Saven A, Burian C, Koziol JA, Piro LD (1998) Longterm follow-up of patients with hairy cell leukemia after cladribine treatment. Blood 92(6):1918–1926

    Article  CAS  Google Scholar 

  12. Flinn IW, Kopecky KJ, Foucar MK, Head D, Bennett JM, Hutchison R et al (2000) Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood 96(9):2981–2986

    CAS  PubMed  Google Scholar 

  13. Foucar KFB, Catovsky D, Stein H (2008) Hairy cell leukaemia. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (eds) WHO classification of tumours of the haematopoietic and lymphoid tissues, 4th edn. IARC Press, Lyon, pp 188–190

    Google Scholar 

  14. Thompson PA, Ravandi F (2017) How I manage patients with hairy cell leukaemia. Br J Haematol 177(4):543–556

    Article  CAS  Google Scholar 

  15. Anonymous. (1987) Consensus resolution: proposed criteria for evaluation of response to treatment in hairy cell leukemia. Leukemia 1:405

  16. Hacioglu S, Bilen Y, Eser A, Sivgin S, Gurkan E, Yildirim R, Aydogdu I, Dogu MH, Yilmaz M, Kayikci O, Tombak A, Kuku I, Celebi H, Akay MO, Esen R, Korkmaz S, Keskin A (2015) Multicenter retrospective analysis regarding the clinical manifestations and treatment results in patients with hairy cell leukemia: twenty-four year Turkish experience in cladribine therapy. Hematol Oncol. 33(4):192–198

    Article  CAS  Google Scholar 

  17. Getta BM, Woo KM, Devlin S, Park JH, Abdel-Wahab O, Saven A, Rai K, Tallman MS (2016) Treatment outcomes and secondary cancer incidence in young patients with hairy cell leukaemia. Br J Haematol 175(3):402–409. https://doi.org/10.1111/bjh.14207

    Article  PubMed  PubMed Central  Google Scholar 

  18. Gupta AK, Sachdeva MU, Ahluwalia J, Das R, Naseem S, Sharma P, Kumar N, Malhotra P, Varma N, Varma S (2015) Haematological profile of 21 patients with hairy cell leukaemia in a tertiary care centre of north India. Indian J Med Res. 142(4):426–429. https://doi.org/10.4103/0971-5916.169204

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Payandeh M, Sadeghi M, Sadeghi E (2015) Hairy cell leukemia: a retrospective study on 11 patients in the Western of Iran. Int J Hematol Oncol Stem Cell Res. 9(3):133–137

    PubMed  PubMed Central  Google Scholar 

  20. Madanat YF, Rybicki L, Radivoyevitch T, Jagadeesh D, Dean R, Pohlman B, Kalaycio M, Sekeres MA, Smith MR, Hill BT (2017) Long-term outcomes of hairy cell leukemia treated with purine analogs: a comparison with the general population. Clin Lymphoma Myeloma Leuk 17(12):857–862

    Article  Google Scholar 

  21. Erkut N, Batur D, Akıdan O, Karabacak V, Sonmez M (2018) Long term treatment outcome of patients with hairy cell leukemia: single center experience. J Med Sci 38(3):236–240

    Google Scholar 

  22. Rosenberg JD, Burian C, Waalen J, Saven A (2014) Clinical characteristics and long-term outcome of young hairy cell leukemia patients treated with cladribine: a single-institution series. Blood 123(2):177–183

    Article  CAS  Google Scholar 

  23. Inbar M, Herishanu Y, Goldschmidt N, Bairey O, Yuklea M, Shvidel L, Fineman R, Aviv A, Ruchlemer R, Braester A, Najib D, Rouvio O, Shaulov A, Greenbaum U, Polliack A, Tadmor T (2018) Hairy cell leukemia: retrospective analysis of demographic data and outcome of 203 patients from 12 medical centers in Israel. Anticancer Res. 38(11):6423–6429

    Article  Google Scholar 

  24. Öngören Ş, Eşkazan AE, Berk S, Elverdi T, Salihoğlu A, Ar MC, Başlar Z, Aydın Y, Tüzüner N, Soysal T (2017) Retrospective evaluation of hairy cell leukemia patients treated with three different first-line treatment modalities in the last two decades: a single-center experience. Turk J Haematol 34(4):291–299

    Article  Google Scholar 

  25. Andrasiak I, Rybka J, Wrobel T (2018) Response to the therapy in hairy cell leukemia: systematic review and meta-analysis. Clin Lymphoma Myeloma Leuk 18(6):392–399.e3

    Article  Google Scholar 

  26. Goodman GR, Burian C, Koziol JA, Saven A (2003) Extended follow-up of patients with hairy cell leukemia after treatment with cladribine. J Clin Oncol 21(5):891–896

    Article  Google Scholar 

  27. Chadha P, Rademaker AW, Mendiratta P, Kim B, Evanchuk DM, Hakimian D, Peterson LC, Tallman MS (2005) Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. Blood 106(1):241–246

    Article  CAS  Google Scholar 

  28. Jehn U, Bartl R, Dietzfelbinger H, Haferlach T, Heinemann V (2004) An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine. Leukemia 18(9):1476–1481

    Article  CAS  Google Scholar 

  29. Maloisel F, Benboubker L, Gardembas M, Coiffier B, Divine M, Sebban C, Blanc M, Abgrall JF, Lederlin P, Harousseau JL, Blaise AM, Grosbois B, Morice P, Ghandour C, Castaigne S (2003) Long-term outcome with pentostatin treatment in hairy cell leukemia patients. A French retrospective study of 238 patients. Leukemia. 17(1):45–51

    Article  CAS  Google Scholar 

  30. Grever M, Kopecky K, Foucar MK, Head D, Bennett JM, Hutchison RE, Corbett WE, Cassileth PA, Habermann T, Golomb H (1995) Randomized comparison of pentostatin versus interferon alfa-2a in previously untreated patients with hairy cell leukemia: an intergroup study. J Clin Oncol 13(4):974–982

    Article  CAS  Google Scholar 

  31. Legrand O, Vekhoff A, Marie JP, Zittoun R, Delmer A (1997) Treatment of hairy cell leukaemia (HCL) with 2-chlorodeoxyadenosine (2-CdA): identification of parameters predictive of adverse effects. Br J Haematol 99(1):165–167

    Article  CAS  Google Scholar 

  32. Hoffman MA, Janson D, Rose E, Rai KR (1997) Treatment of hairy-cell leukemia with cladribine: response, toxicity, and long-term follow-up. J Clin Oncol 15(3):1138–1142

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Hematology, Internal Medicine, Marmara University, Istanbul, Turkey

    Fergün Yılmaz

  2. Department of Hematology, Internal Medicine, Ege University, Izmir, Turkey

    Dilan Atilla, Hale Bülbül, Nur Soyer, Filiz Vural, Güray Saydam & Fahri Şahin

  3. İnternal Medicine, Department of Hematology, İzmir Katip Celebi University, Izmir, Turkey

    Nagihan Akkaş, Demet Kiper & Bahriye Payzın

  4. Department of Pathology, Ege University, Izmir, Turkey

    Derya Demir, Mine Hekimgil & Nazan Özsan

  5. Department of Pathology, İzmir Katip Celebi University, Izmir, Turkey

    Aylin Avcı

  6. Department of Public Heath, Ege University, Izmir, Turkey

    Raika Durusoy

Authors
  1. Fergün Yılmaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Dilan Atilla
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Nagihan Akkaş
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hale Bülbül
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Nur Soyer
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Derya Demir
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Demet Kiper
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Aylin Avcı
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Filiz Vural
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Güray Saydam
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Fahri Şahin
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Mine Hekimgil
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Nazan Özsan
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Raika Durusoy
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Bahriye Payzın
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Fergün Yılmaz.

Ethics declarations

Conflict of interest

Authors declare that they have no conflict of interest.

Informed Consent

Informed consent was obtained from all participants included in the study.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Yılmaz, F., Atilla, D., Akkaş, N. et al. Retrospective Analysis of Hairy Cell Leukemia Patients Treated with Different Modalities as First Line: Real-Life Experience Over 20 years. Indian J Hematol Blood Transfus 35, 692–698 (2019). https://doi.org/10.1007/s12288-019-01132-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12288-019-01132-7

Keywords

Search

Navigation