Hairy cell leukemia - clinical profile and treatment outcome from a Tertiary Regional Cancer Institute in South India

摘要

Background: Hairy cell leukemia (HCL) is an indolent neoplasm of small mature B lymphoid cells. It is characterized by pancytopenia, splenomegaly, bone marrow fibrosis, and presence of atypical lymphoid cells with hairy projections in peripheral blood, bone marrow and spleen. HCL is potentially curable and treatment with purine analog cladribine induces complete remission (CR). Materials and Methods: This is a retrospective analysis of 10 HCL cases diagnosed in the Department of Medical Oncology at a Tertiary Regional Cancer Institute, South India, over 7 years. The clinical features, laboratory parameters, bone marrow findings, cytochemistry, immunophenotyping, and outcome with treatment were studied. Results: Among 8 cases of HCL who were treated with cladribine, 7 achieved remission and 1 succumbed to infection during course of treatment. Median overall survival in these 7 cases was 61 months. Conclusion: HCL is a chronic lymphoproliferative neoplasm with potentially curative treatment. Cladribine is treatment of choice and majority of patients achieve long-lasting CR. Upon relapse, these patients can be successfully salvaged with cladribine retreatment.