T-cell prolymphocytic leukemia

Abstract

T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell lymphoma. The diagnosis of T-PLL is made based on the criteria proposed in 2019 by the T-PLL International Study Group (TPLL-ISG). T-PLL may be diagnosed in an ‘active’ or an ‘inactive’ form. While T-PLL is most often characterized by an aggressive clinical course, approximately 20–30% of patients may have a stable or slowly progressive disease in the initial period. Only the active form requires treatment. The treatment initiation criteria were defined in the consensus proposed by the TPLL-ISG. Data on the effectiveness of various therapeutic approaches comes from small, non-randomized studies. No drug is approved for the treatment of T-PLL. The standard of care in the first line of treatment is intravenous alemtuzumab. Consolidation therapy in eligible patients consists of allogeneic hematopoietic stem cell transplantation, preferably after TBI-based conditioning. In the remaining patients, neither maintenance nor consolidation treatment is recommended. There is no standard therapy for the relapse, although, based on genome studies, many drugs may be potentially effective, including histone deacetylase inhibitors and BCL2 inhibitors.