title: Effective treatment with rituximab in a patient with refractory prolymphocytoid transformed B-chronic lymphocytic leukemia and Evans syndrome.
abstract: A 65-year-old male who had been diagnosed as having chronic lymphocytic leukemia (CLL) stage Rai 0 three years previously presented in October 1998 with progressive disease (splenomegaly, increasing lymphocytosis: 30/nl) and 50% prolymphocytes in his peripheral blood. The patient was refractory to initial treatment with fludarabine and epirubicin, and 2-chlorodeoxyadenosine. Additionally, the patient developed Evans syndrome. Therapy with cyclophosphamide, vincristine, and prednisone lead to an improvement of hemolysis, but the patient remained thrombocytopenic. Subsequently, lymphocytes further increased to 135/nl, and cyclophosphamide 3 g/m2 was again given but without effect on the peripheral lymphocyte count. At this stage, therapy with rituximab 375 mg/m2 four times weekly was initiated. The platelet count normalized within 1 week after the first dose of rituximab, and the lymphocyte count dropped within 2 months to 1.1/nl. This case demonstrates that patients with prolymphocytoid transformed B-CLL refractory to purine analogs and alkylating agents and autoimmune-hemolytic disease can effectively and safely be treated with rituximab and indicates that an investigation of antibody therapy is warranted in this patient group.
authors.affiliation: Medizinische Klinik III, Hämatologie/Onkologie, Universitätsklinik Frankfurt, Theodor-Stern Kai 7, 60590 Frankfurt/Main, Germany. G.Seipelt@em.uni-frankfurt.de
authors.forename: G
authors.initials: G
authors.lastname: Seipelt
authors.forename: A
authors.initials: A
authors.lastname: Böhme
authors.forename: S
authors.initials: S
authors.lastname: Koschmieder
authors.forename: D
authors.initials: D
authors.lastname: Hoelzer
mesh_terms: D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D000069461:Bendamustine Hydrochloride; D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D009588:Nitrogen Mustard Compounds / Q000627:therapeutic use; D012074:Remission Induction; D000069283:Rituximab; D017211:Treatment Failure
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D009588:Nitrogen Mustard Compounds; D000069283:Rituximab; D000069461:Bendamustine Hydrochloride