Abstract
T-cell prolymphocytic leukemia (T-PLL) is a rare, aggressive hematologic malignancy with a poor prognosis. Alemtuzumab (Campath) remains the cornerstone for treatment, with an 80% complete response (CR). Hematopoietic stem cell transplant (HSCT) is considered the standard of care as consolidative therapy in eligible patients. However, allogeneic stem cell transplant is also complicated by increased rates of infections from chemotherapy, acute graft-versus-host disease (GVHD), and chronic GVHD. This review aims to report the available literature on the efficacy and complications of consolidative HSCT. It also discusses the importance of patient selection and pre- and post-transplant complications including atypical infections and GVHD.
Highlights
T-cell prolymphocytic leukemia (T-PLL) is a rare aggressive malignancy originating from the mature post-thymic T cell
Anti-CD52 antibody, Campath, as a single agent given intravenously remains the standard of care for induction therapy in T-PLL
Despite high OR rate (ORR), the complete response (CR) is short-lived; and stem cell consolidation therapy is essential to provide an opportunity for cure [6]
Summary
T-cell prolymphocytic leukemia (T-PLL) is a rare aggressive malignancy originating from the mature post-thymic T cell. High expression of CD52 provides an effective therapeutic approach for these patients with Campath (alemtuzumab), an anti-CD52 monoclonal antibody that has robust activity in newly diagnosed and recurrent T-PLL [5, 6]. Targeted therapy with inhibition of HiDAC (Histone Deacetylase), BCL2 (B-Cell Lymphoma-2), and JAK-STAT (Janus Kinases, Signal Transducer and Activator of Transcription proteins) have shown to be very promising in Phase I and preclinical studies [9, 10]. Despite multiple therapeutic options that are currently being studied, the current standard of care is a consolidative allogeneic stem cell transplant following induction therapy with Campath in transplant-eligible patients [11,12,13]. Further collaborative studies combining these therapeutic modalities are needed to improve prognosis and OS
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