, Volume 92, Issue 5, pp 711-713
Date: 25 Nov 2012

Splenectomy followed by administration of rituximab is useful to treat a patient with hairy cell leukemia-variant

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Dear Editor,

Hairy cell leukemia-variant (HCL-v) is a rare disease-category separated from hairy cell leukeimia (HCL) by the WHO classification [1]. The prognosis, characterized by splenomegaly, is relatively poor among splenomegalic B cell disorders including HCL and splenic marginal zone lymphoma (SMZL) [2]. Thus, a novel therapeutic modality has been explored for HCL-v patients.

Here, we show that splenectomy followed by rituximab monotherapy led to molecular complete response (CR) with an undetectable level of PCR-based immunoglobulin heavy-chain gene (PCR-based IgH) rearrangement in a patient with HCL-v.

A 67-year-old female presented with an abdominal mass. Computed tomography showed significant splenomegaly (Fig. 1a). Pancytepenia (WBC count, 1.88 × 109/μl; Hb, 10.8 g/dl; and platelet count, 121 × 109/μl) and atypical lymphocytes (4.0 %) were noted in peripheral blood. Hepatitis B virus antigen and hepatitis C virus antibody were negative. Soluble interleukin-2 receptor was 835 IU/ ...