Qasim Mahmood Buttar, Rizwan Aziz Qazi, Sohail Athar, Sadia Noor.
Clinicopathological Features and Survival Outcome in Patients with Hairy Cell Leukemia (HCL) treated with Cladribine; A Single Institution Experience.
Ann Pak Inst Med Sci Jan ;10(3):136-9.

Background: Hairy cell leukemia (HCL) is a rare chronic indolent B cell disorder. It is characterized by pancytopenia, splenomegaly and the presence of Hairy cell in peripheral blood and Bone Marrow. After the introduction of Purine analogues, treatment of HCL has been revolutionized. Methods: In this Retrospective Analysis, 22 patients with HCL were included from 2004 to 2014 treated in Medical Oncology Department at Pakistan Institute of Medical Sciences Islamabad. Their baseline characteristics and type of treatment were recorded. Results: Total number of patient was 22 ; 21 (95.5%) patients were male and Median age was 45 years (range 26-72). Most common presentation was Cytopenias with 18 patients (81.8%) had Thrombocytopenia, 17 (77.3%) patients had Anemia (Hb <11g/dl), Eleven patients (50%) having Leucopenia , while 2 patient (9.1%) had leukocytosis. Monocytopenia was found in 19 patients (86.3%). All patients had Splenomegaly at presentation with mean splenic size of 11.0 cm. All Bone Marrow specimens were TRAP Positive. Seventeen patients (77.3%) were treated with Cladarbine, 02 patients (9.1%) had splenectomy done, 01 patient (4.5%) was treated with Interferon, 01 patient (4.5%) was on observation and 01 patient (4.5%) was not offered treatment. Out of 22 patients 19 patients (86.4%) are alive and 03 patients (13.6%) died. Main cause of death was sepsis. Complete response (CR) was achieved in 14 patients (82.3%) treated with cladribine. Table 1 Median follow-up was 6.3 years (range 0.2-10.0 years). Median Survival for all patients was not reached. Kaplan Meier estimated overall survival (OS) at 10 years to be 72.7%. (Fig 1) Conclusion:HCL is uncommon B cell disorder with good clinical outcome when treated with Cladribine.

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