Advances in the understanding and management of T-cell prolymphocytic leukemia

// Kamel Laribi 1 , Pierre Lemaire 2 , Jeremy Sandrini 3 and Alix Baugier de Materre 4 1 Department of Hematology, Centre Hospitalier du Mans, Le Mans, France 2 Laboratory of Biology and Hematology, Centre Hospitalier du Mans, Le Mans, France 3 Laboratory of Anatomopathology, Centre Hospitalier du Mans, Le Mans, France 4 Department of Medicine, Clinique du Pre, Le Mans, France Correspondence to: Kamel Laribi, email: klaribi@ch-lemans.fr Keywords: T-cell prolymphocytic leukemia, morphology, cytogenetic, molecular biology, treatment Received: February 13, 2017      Accepted: August 27, 2017      Published: November 01, 2017 ABSTRACT T-prolymphocytic leukemia (T-PLL) is a rare T-cell neoplasm with an aggressive clinical course. Leukemic T-cells exhibit a post-thymic T-cell phenotype (Tdt − , CD1a − , CD5 + , CD2 + and CD7 + ) and are generally CD4 + /CD8 − , but CD4 + /CD8 + or CD8 + /CD4 − T-PLL have also been reported. The hallmark of T-PLL is the rearrangement of chromosome 14 involving genes for the subunits of the T-cell receptor (TCR) complex, leading to overexpression of the proto-oncogene TCL1. In addition, molecular analysis shows that T-PLL exhibits substantial mutational activation of the IL2RG-JAK1-JAK3-, STAT5B axis. T-PLL patients have a poor prognosis, due to a poor response to conventional chemotherapy. Monoclonal antibody therapy with antiCD52-alemtuzumab has considerably improved outcomes, but the responses to treatment are transient; hence, patients who achieve a response to therapy are considered for stem cell transplantation (SCT). This combined approach has extended the median survival to four years or more. Nevertheless, new approaches using well-tolerated therapies that target growth and survival signals are needed for most patients unable to receive intensive chemotherapy.