LEUCÉMIE PROLYMPHOCYTAIRE DE PHÉNOTYPE B DE MAUVAIS PRONOSTIC

Abstract

Introduction : B cell prolymphocytic leukemia is a rare disease with poor prognosis compared with chronic lymphocytic leukemia. Case report : Patient aged 75 years old, diabetic and hypertensive, with a massive splenomegaly associated with leukocytosis exceeding 100000/mm3 predominantly prolymphocytic, invading the bone marrow. Immunophenotyping showed CD19 +, CD20 +, CD22 +, CD5 part, CD23+ CD79b + FMC7 stronger and stronger, Smg + in vafour for a type B-cell prolymphocytic leukemia. The evolution was rapidly unfavorable despite an first line treatment of eight cycles of COP (cyclophosphamide, vincristine and prednisone). Partial remission was short, followed by the reappearance of the splanomegaly, new increase in the of Prolymphocyte tate with thrombocytopenia and profound anemia. The patient had received two courses of Fludarabine. Dath occurred after eighteen months of follow up due to a state of septic shock and tumor lysis syndrome. Discussion : This malignancy accounts for 2% of all chronic lymphocytic leukemia with 80% phenotype B. It frequently affects patients of 70 years old with a clear male predominance. The prognosis is more severe than that of chronic lymphocytic leukemia. Anemia 100000/mm3 are the factors of poor prognosis. The median survival was 65 months reported by Shvidel et al. in a series of 35 observations. Conclusion : Promising results are reported with new treatments. The antibady anti-CD20 has been used successfully for the second type of B-cell prolymphocytic leukemia after relapse. The allograft or autograft are indicated, but it is mostly used in patients, who have contraindications to a heavy treatment.