Outcome of hairy cell leukemia patients treated with cladribine - a 10-year single-center experience in Pakistan

Zahid, Mohammad Faizan; Mehdi, Mohammad Qasim; Ali, Natasha

doi:10.1016/j.htct.2018.08.006

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Hematology, Transfusion and Cell Therapy

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Original Articles • Hematol., Transfus. Cell Ther. 41 (2) • Apr-Jun 2019 • https://doi.org/10.1016/j.htct.2018.08.006 copy

Outcome of hairy cell leukemia patients treated with cladribine - a 10-year single-center experience in Pakistan

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Introduction and objective:

Hairy cell leukemia is an uncommon, indolent B-cell lymphoproliferative disorder. Therapy with cladribine (2-chlorodeoxyadenosine) is able to induce complete remission (CR) in the majority of patients after a single course of treatment. We report the outcomes of patients treated at Aga Khan University Hospital, Karachi, Pakistan.

Methods:

This was a retrospective review. Medical records of patients were used to collect data.

Results:

A total of 21 patients with hairy cell leukemia were treated with cladribine. All patients achieved an initial CR. Four patients (19%) required hospitalization and therapy for neutropenic fever. Six patients (29%) relapsed at a median of 48 months. All 6 patients were treated for relapse, out of which 4 achieved CR, 1 had partial response and 1 had refractory disease. The overall survival rate was 90.5%, with a median follow-up of 35 months.

Conclusion:

A single course of cladribine is able to induce CR in a vast majority of patients. Unfortunately, relapse is not uncommon. Patients who relapse can be successfully retreated with cladribine. Cladribine has impressive efficacy and a favorable acute and long-term toxicity profile when administered to patients with HCL.

Keywords:
Hairy cell leukemia; Outcome; Cladribine; 2-Chlorodeoxyadenosine

Characteristics	n (%)
Male gender	21 (100)
Median age (range), years	52 (31-75)

Initial symptoms
Fever	16 (76)
Fatigue	9 (43)
Symptomatic splenomegaly	8 (38)
Weight loss	4 (19)
Marrow failure	4 (19)^a a 2 patients had recurrent infections, 1 had pallor and fatigue, 1 had recurrent bleeding.
Other	6 (29)^b b 3 patients had knee joint pain, 2 had generalized body aches, 1 had generalized lymphadenopathy.

Peripheral blood counts at diagnosis
Hemoglobin, median (range), g/dL	9.1 (6.7-19.4)
WBC count, median (range), ×10⁹/L	6.0 (0.7-53.1)
ANC, median (range), ×10⁹/L	0.8 (0.02-4.6)
Platelets, median (range), ×10⁹/L	56 (24-185)
Median bone marrow infiltration by hairy cells (range), %	49 (14-78)

Disease type
Classical HCL	20 (95)
Variant HCL	1 (5)
Median time from diagnosis to 2CdA therapy (range), months	1 (0-11)
Median follow up (range), months	35 (1-182)

Patient number	Time of relapse after initial cladribine therapy, months	Treatment of relapse	Neutropenic fever	Outcome of relapse treatment	Second relapse	Treatment of second relapse	Final outcome
1	9	2CdA	No	CR	Yes	None	Alive with active disease
2^a a Treated with 2CdA 0.09 mg/kg/day over 7 days.	37	2CdA	Yes - A. fumigatus pneumonia twice treated with amphotericin B	CR			CR
3	40	Radiation	No	CR			CR
4	55	2CdA	No	PR			Alive with active disease
5^a a Treated with 2CdA 0.09 mg/kg/day over 7 days.	74	Interferon	Yes - unknown source of infection	Refractory disease			Died
6	104	2CdA	No	CR			CR

Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH) R. Dr. Diogo de Faria, 775 cj 133, 04037-002, São Paulo / SP - Brasil - São Paulo - SP - Brazil
E-mail: htct@abhh.org.br

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[1] *Corresponding author at: Department of Pathology and Laboratory Medicine/Oncology, The Aga Khan University Hospital, P.O Box 3500, Stadium Road, Karachi 74800, Pakistan. E-mail address: natasha.ali@aku.edu (N. Ali).