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

Kamel Laribi _, Pierre Lemaire, Jeremy Sandrini and Alix Baugier de Materre

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Oncotarget. 2017; 8:104664-104686. https://doi.org/10.18632/oncotarget.22272

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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

Correspondence to:

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.

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