Effective treatment with rituximab in a patient with refractory prolymphocytoid transformed B-chronic lymphocytic leukemia and Evans syndrome

A 65-year-old male who had been diagnosed as having chronic lymphocytic leu kemia (CLL) stage Rai 0 three years previously presented in October 1998 wi th progressive disease (splenomegaly, increasing lymphocytosis: 30/nl) and 50% prolymphocytes in his peripheral blood. The patient was refractory to i nitial treatment with fludarabine and epirubicin, and 2-chlorode-oxyadenosi ne. Additionally, the patient developed Evans syndrome. Therapy with cyclop hosphamide, vincristine, and prednisone lead to an improvement of hemolysis , but the patient remained thrombocytopenic. Subsequently, lymphocytes furt her increased to 135/nl, and cyclophosphamide 3 g/m(2) was again given but without effect on the peripheral lymphocyte count. At this stage, therapy w ith rituximab 375 mg/m(2) four times weekly was initiated. The platelet cou nt normalized within 1 week after the first dose of rituximab, and the lymp hocyte count dropped within 2 months to 1.1/nl. This case demonstrates that patients with prolymphocytoid transformed B-CLL refractory to purine analo gs and alkylating agents and autoimmune-hemolytic disease can effectively a nd safely be treated with rituximab and indicates that an investigation of antibody therapy is warranted in this patient group.