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Adult T Cell Leukemia-Lymphoma (ATL): State of the Art

  • T-Cell and Other Lymphoproliferative Malignancies (J Zain, Section Editor)
  • Published:
Current Hematologic Malignancy Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

ATL is a rare and highly aggressive T cell malignancy caused by HTLV-1. We will review the state of the art of ATL epidemiology, pathogenesis, diagnosis, and treatment.

Recent Findings

Because of population migration, cases of ATL in non-HTLV-1 endemic countries including North America and Europe are increasingly recognized. ATL has diverse clinical manifestations, limited treatment options not widely available globally, and poor prognosis despite therapy. A small subset of patients may achieve prolonged survival with antiviral therapy or allogeneic stem cell transplant. Mogamulizumab, an anti-CCR4 monoclonal antibody, and lenalidomide, an immunomodulatory drug, are approved for ATL in Japan. Molecular studies have identified key alterations in T cell signaling and DNA methylation that may further guide drug development. Patients should be encouraged to enroll in prospective clinical trials when possible.

Summary

Ongoing, collaborative, international research continues to elucidate disease pathogenesis and contribute to an evolving therapeutic landscape for ATL.

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Authors and Affiliations

  1. Department of Medicine, Weill Cornell Medical College, New York, NY, 10065, USA

    Adrienne A. Phillips

  2. Division of Hematology and Oncology, Weill Cornell Medical College, 525 East 68th Street Starr Pavilion, 3rd Floor, New York, NY, 10065, USA

    Adrienne A. Phillips

  3. Department of Medicine, New York Presbyterian Queens, Flushing, NY, 11355, USA

    Janine C. K. Harewood

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Correspondence to Adrienne A. Phillips.

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Conflict of Interest

Adrienne Phillips is a principal investigator for a prospective, multicenter, randomized study of anti-CCR4 monoclonal antibody mogamulizumab (moga) vs investigator’s choice (IC) in the treatment of patients (pts) with relapsed/refractory (R/R) adult T cell leukemia-lymphoma (ATL) and received research funding. Janine Harewood declares that she has no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

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Phillips, A.A., Harewood, J.C.K. Adult T Cell Leukemia-Lymphoma (ATL): State of the Art. Curr Hematol Malig Rep 13, 300–307 (2018). https://doi.org/10.1007/s11899-018-0458-6

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