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Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment

  • Leukemia (A Aguayo, Section Editor)
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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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    Authors and Affiliations

    1. Department of Hematology, Institut Paoli-Calmettes, 232 Bd Ste Margerite, 13009, Marseille, France

      Aude Collignon, Anne Wanquet & Thérèse Aurran-Schleinitz

    2. Centre Hospitalo-universitaire de Caen, Caen, France

      Elsa Maitre, Edouard Cornet & Xavier Troussard

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    1. Aude Collignon
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    2. Anne Wanquet
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    3. Elsa Maitre
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    Correspondence to Thérèse Aurran-Schleinitz.

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    Aude Collignon, Anne Wanquet, Elsa Maitre, Edouard Cornet, Xavier Troussard, and Thérèse Aurran-Schleinitz declare that they have no conflict of interest.

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    This article is part of the Topical Collection on Leukemia

    Aude Collignon, Anne Wanquet and Elsa Maitre contributed equally to this work.

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    Collignon, A., Wanquet, A., Maitre, E. et al. Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment. Curr Oncol Rep 19, 29 (2017). https://doi.org/10.1007/s11912-017-0581-x

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