B-cell prolymphocytic leukemia (B-PLL) and T-cell prolymphocytic leukemia (T-PLL)

Abstract

Both prolymphocytic leukemia of T-cell (T-PLL) and B-cell (B-PLL) phenotype are rare and aggressive malignancies characterized by marked lymphocytosis and splenomegaly. Given the poor response to conventional therapy including alkylating agents or purine analogues, overall survival in most cases is less than 12 months. Monoclonal antibodies, e.g., rituximab in B-PLL and alemtuzumab in B-PLL and T-PLL, demonstrated encouraging activity in terms of response. However, long-term survival is rare, and allogeneic transplant might be considered as an option in selected patients.