Adult T Cell Leukemia-Lymphoma (ATL): State of the Art
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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.
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.
Ongoing, collaborative, international research continues to elucidate disease pathogenesis and contribute to an evolving therapeutic landscape for ATL.
KeywordsHTLV-1 ATL Non-Hodgkin’s lymphoma
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.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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