Advances in the understanding and management of T-cell prolymphocytic leukemia
Metrics: PDF 2233 views | HTML 7154 views | ?
Kamel Laribi1, Pierre Lemaire2, Jeremy Sandrini3 and Alix Baugier de Materre4
1Department of Hematology, Centre Hospitalier du Mans, Le Mans, France
2Laboratory of Biology and Hematology, Centre Hospitalier du Mans, Le Mans, France
3Laboratory of Anatomopathology, Centre Hospitalier du Mans, Le Mans, France
4Department of Medicine, Clinique du Pré, Le Mans, France
Kamel Laribi, email: [email protected]
Keywords: T-cell prolymphocytic leukemia, morphology, cytogenetic, molecular biology, treatment
Received: February 13, 2017 Accepted: August 27, 2017 Published: November 01, 2017
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.
All site content, except where otherwise noted, is licensed under a Creative Commons Attribution 4.0 License.