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Current Oncology Reports
Published in: Current Oncology Reports 4/2017

01-04-2017 | Leukemia (A Aguayo, Section Editor)

Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment

Authors: Aude Collignon, Anne Wanquet, Elsa Maitre, Edouard Cornet, Xavier Troussard, Thérèse Aurran-Schleinitz

Published in: Current Oncology Reports | Issue 4/2017

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Abstract

Purpose of Review

We aimed to produce a comprehensive update on clinical and biological data regarding two rare lymphoid neoplasms, B and T prolymphocytic leukemias, and assess therapeutic management in the light of new molecular insights and the advent of targeted therapies.

Recent Findings

B cell prolymphocytic leukemia (B-PLL) diagnosis remains challenging in the absence of clear immunophenotypic or cytogenetic signature and overlap with mantle cell lymphoma. New molecular defects have been identified in T cell prolymphocytic leukemia (T-PLL), especially in the JAK STAT pathway. Like in chronic lymphocytic leukemia (CLL), B-PLL treatment depends on the presence of TP53 dysfunction. In T-PLL, alemtuzumab still remains the standard of care. Allogeneic transplantation is the only curable option. Thanks to reduced intensity conditioning regimens, it has become accessible to a larger number of patients.

Summary

PLL prognosis remains poor with conventional therapies. However, great advances in the understanding of both T- and B-PLL pathogenesis lead to promising new therapeutic agents.
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Metadata
Title
Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment
Authors
Aude Collignon
Anne Wanquet
Elsa Maitre
Edouard Cornet
Xavier Troussard
Thérèse Aurran-Schleinitz
Publication date
01-04-2017
Publisher
Springer US
Published in
Current Oncology Reports / Issue 4/2017
Print ISSN: 1523-3790
Electronic ISSN: 1534-6269
DOI
https://doi.org/10.1007/s11912-017-0581-x

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