TREATMENT OUTCOMES OF PATIENTS WITH HAIRY CELL LEUKAEMIA; A 16-YEAR EXPERIENCE AT A TERTIARY CARE CENTER IN PAKISTAN

Authors

  • Muhammad Yousaf Armed forces of Bone Marrow transplant center
  • Mehreen Ali Khan 1. Department of Hematology and Stem Cell Transplant, Armed Forces Bone Marrow Transplant Center/National Institute of Blood and Marrow Transplant, Rawalpindi Pakistan, 46000
  • Raheel Iftikhar Department of Hematology and Stem Cell Transplant, Armed Forces Bone Marrow Transplant Center/National Institute of Blood and Marrow Transplant, Rawalpindi Pakistan, 46000
  • Qamar Un Nisa Chaudary Department of Hematology and Stem Cell Transplant, Armed Forces Bone Marrow Transplant Center/National Institute of Blood and Marrow Transplant, Rawalpindi Pakistan, 46000
  • Nighat Shahbaz Department of Hematology and Stem Cell Transplant, Armed Forces Bone Marrow Transplant Center/National Institute of Blood and Marrow Transplant, Rawalpindi Pakistan, 46000
  • Uzair Ahmad Department of Hematology and Stem Cell Transplant, Armed Forces Bone Marrow Transplant Center/National Institute of Blood and Marrow Transplant, Rawalpindi Pakistan, 46000
  • Hammad Javed Department of Hematology and Stem Cell Transplant, Armed Forces Bone Marrow Transplant Center/National Institute of Blood and Marrow Transplant, Rawalpindi Pakistan, 46000

DOI:

https://doi.org/10.55519/JAMC-04-10177

Keywords:

Hairy cell leukemia, Outcome, cladribine

Abstract

Background: Hairy cell leukaemia (HCL) is an uncommon neoplasm of mature B-lymphoid cells which is characterized by cytopenias, commonly of all three cell lines, with typical hairy cells on peripheral smear and/or bone marrow along with organomegaly. Objective was to document the outcomes of HCL patients treated at a tertiary care hospital in Pakistan. Methods: Medical records of patients from 2004 to 2020 were reviewed and data was collected to assess patient’s demographics, symptomatology, remission rate and overall survival. The record flies of all patients presenting to AFBMTC with HCL were included in the study. The record file with insufficient data were excluded Results: 26 patients with a mean age of 48.12±11.43 years were diagnosed with HCL and treated at AFBMTC. Out of these, 23 (88.4%) were male and 03 (11.5%) females. The main presenting complaints were generalized body aches (34.6%), fever (15.4%), incidental finding of cytopenias (11.5%) and abdominal discomfort (26.9%). Splenomegaly was found in 76.92% while hepatomegaly was found in 46.15% of patients. A total of 12 (46.15%) patients received Cladribine (either intravenous or subcutaneous) and splenectomy was done in 7 (26.92%) as 1st line treatment. Eleven patients out of 12 (83.33%) who received Cladribine and 05 (71.42%) patients out of seven who underwent splenectomy; achieved complete remission (CR) after 1st line of treatment. One patient received Cladribine as 1st line of treatment but did not respond and CHOP regimen was given as second line. Out of the 26 patients, 5 patients (19.23%) relapsed at a median interval of 5.83±6.6 years. Two patients received Cladribine + Rituximab while 03 patients received cladribine as their salvage therapy. Disease free survival (DFS) of 71.4% among the patients underwent splenectomy while 75.0% among the patients received Cladribine. DFS for combination therapy (included CHOP and CVP) was 66.7% while OS was calculated among patients who received cladribine, splenectomy and combination chemotherapy as 100%, 85.7%, 66.7% respectively. Conclusion: Cladribine has a significant efficacy and encouraging acute and long-term benefits when administered to patients with HCL. A single course of cladribine was able to induce CR in a vast majority of patients. At a median follow up of 4.6 years the OS was 100% with cladribine and 85% with splenectomy. Those who relapsed were successfully retreated with cladribine + Rituximab.

References

Zahid MF, Mehdi MQ, Ali N. Outcome of hairy cell leukemia patients treated with cladribine - a 10-year single-center experience in Pakistan. Hematol Transfus Cell Ther 2019;41(2):134–8.

Getta BM, Woo KM, Devlin S, Park JH, Abdel-Wahab O, Saven A. Treatment outcomes and secondary cancer incidence in young patients with hairy cell leukaemia. Br J Haematol 2016;175(3):402–9.

Yam LT, Li CY, Lam KW. Tartrate-resistant acid phosphatase isoenzyme in the reticulum cells of leukemic reticuloendotheliosis. N Engl J Med 1971;284(7):357–60.

Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S et.al. Long‐term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. British journal of haematology. 2016;174(5):760–6.

Yılmaz F, Atilla D, Akkaş N, Bülbül H, Soyer N, Demir D, 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 2019;35(4):692–8.

Bohn JP, Salcher S, Pircher A, Untergasser G, Wolf D. The Biology of Classic Hairy Cell Leukemia. Int J Mol Sci 2021;22(15):7780.

Grever MR, Abdel-Wahab O, Andritsos LA, Banerji V, Barrientos J, Blachly JS, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood 2017;129(5):553–60

Thompson PA, Ravandi F. How I manage patients with hairy cell leukaemia. Br J Haematol 2017;177(4):543–56

Quesada JR, Reuben J, Manning JT, Hersh EM, Gutterman JU. Alpha interferon for induction of remission in hairy-cell leukemia. N Engl J Med 1984;310(1):15–8.

Levy I, Tadmor T. Time to Cure Hairy Cell Leukemia. Tüylü Hücreli Löseminin Kür Edilme Zamanı. Turk J Haematol 2017;34(4):289–90.

Paillassa J, Cornet E, Noel S, Tomowiak C, Lepretre S, Vaudaux 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.

Vemurafenib plus rituximab in refractory or relapsed hairy-cell leukemia. et al. Vemurafenib plus rituximab in refractory or relapsed hairy-cell leukemia. N Engl J Med 2021;384(19):1810–23.

Chihara D, Arons E, Stetler-Stevenson M, Yuan CM, Wang HW, Zhou H, et al. Randomized phase II study of 1st-line cladribine with concurrent or delayed rituximab in patients with hairy cell leukemia. J Clin Oncol 2021;38(14):1527–38.

Wierda WG, Byrd JC, Abramson JS, Bhat S, Bociek G, Brander D, et al. Hairy cell leukemia, Version 2.2018, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw 2017;15(11):1414–27.

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;131(12):1380–3.

Rosenberg JD, Burian C, Waalen J, Saven A. Clinical characteristics and long-term outcome of young hairy cell leukemia patients treated with cladribine: a single-institution series. Blood 2014;123(2):177–83.

Öngören Ş, Eşkazan AE, Berk S, Elverdi T, Salihoğlu A, Ar MC, et al. Retrospective evaluation of hairy cell leukemia patients treated with 3 different first-line treatment modalities in the last two decades: a single center experience. Turk J Haematol 2017;34(4):291–99.

Wierda WG, Byrd JC, Abramson JS, Bhat S, Bociek G, Brander D, et al. Hairy cell leukemia, Version 2.2021 NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw 2017;15(11):1414–27.

Jameel A, Chiragh S, Iqbal S, Farooq M. Clinico-pathological presentation and therapeutic options for hairy cell leukemia. J Postgr Med Inst 2012;26(3):336–9.

Kreitman RJ, Arons E. Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues. Blood Rev 2022;51:100888.

da Silva WF, Neto AC, da Rosa LI, de Siqueira IA, Amarante GD, Velloso EDRP, et al. Outcomes and second neoplasms in hairy cell leukemia: A retrospective cohort. Leuk Res 2019;83:106–65.

Published

2022-09-27

Issue

Vol. 34 No. 4 (2022): JOURNAL OF AYUB MEDICAL COLLEGE ABBOTTABAD

Section

ORIGINAL ARTICLE

License

Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License  CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.

USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.

AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.

Most read articles by the same author(s)