Hairy cell leukemia : experience at a tertiary cancer centre in Northern India

Abstract

Background : The prognosis of Hairy cell leukemia (HCL) has improved markedly following treatment with cladribine (2- CdA), a nucleoside analogue. We reviewed data on patients with HCL treated in our department. Methods . Between 1995 and 2004, 23 patients with hairy cell leukemia (HCL) were diagnosed Patients median age was 48.5 years (range, 32 to 66 years), there were 18 males and 5 females. The common presenting symptoms were: fatigue (82.6%), fever (34.7%), abdominal discomfort (21.7%), arthralgia/bone pains (13%) and bleeding (17.3%). 22 of 23 (95.6%) patients had enlarged spleen, and hepatomegaly (65%). Lymph node enlargement was present in 17% of patients. Investigations revealedmedian Hb of 7.8g% (5.7 to 12.9 g%), thrombocytopenia (median 55000/cmm) and median WBC count of 3500/cmm (range, 600 to 20,200/cmm). Bicytopenia or pancytopenia was present in 87% and bone marrow fibrosis in 75% of cases. Immunophenotyping studies revealed expression of CD11C (60%), CD25 (60%), FMC7(47.8%), CD23(34.8%), CD103 in 39% of cases. 20 of 23 patients received treatment; two received treatment else where and one patient died of liver failure prior to treatment. 17 of 20 patients were treated with 2-CdA, 2 with interferon alfa (IFN-a) and one patient underwent splenectomy alone. Three patients received 2-CdA as second line therapy for treatment of relapse: this includes . one patient each, treated with IFN- α and splenectomy both and one patient received 2-cdA twice in view of relapse. Results . Following treatment with 2CdA . 95% of patients responded; complete-84.2% and partial response in 10.5%. one (5.2%) patient died of toxicity. Two patients relapsed at a mean follow up of 25 months. The common side effects were febrile episodes (n=10) and grade I-II myelosuppression. One patient died of toxicity to 2-CdA. This patient was a known case of multidrug resistant disseminated tuberculosis. He had severe myelosuppression with fungal sepsis with multiorgan failure and BM Aspirate was positive for AFB. Other infections documented were Pulmonary tuberculosis in 2 and herpes zoster in one patient. The patient who underwent Splenectomy achieved remission but relapsed after 3 years and was salvaged with 2-CdA again. Median time for normalization of blood counts after 2-CdA was 28 days and median time to regress spleen was 41.5 days . Conclusion . Present study confirms good outcome with 2-CdA (cladribine) therapy for patients of hairy cell leukemia.