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Current Hematologic Malignancy Reports

, Volume 13, Issue 4, pp 300–307 | Cite as

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

  • Adrienne A. Phillips
  • Janine C. K. Harewood
T-Cell and Other Lymphoproliferative Malignancies (J Zain, Section Editor)
Part of the following topical collections:
  1. Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

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.

Keywords

HTLV-1 ATL Non-Hodgkin’s lymphoma 

Notes

Compliance with Ethical Standards

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.

Human and Animal Rights and Informed Consent

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

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Adrienne A. Phillips
    • 1
    • 2
  • Janine C. K. Harewood
    • 3
  1. 1.Department of MedicineWeill Cornell Medical CollegeNew YorkUSA
  2. 2.Division of Hematology and OncologyWeill Cornell Medical CollegeNew YorkUSA
  3. 3.Department of MedicineNew York Presbyterian QueensFlushingUSA

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