Table of Contents
Leukemia Research and Treatment
Volume 2012 (2012), Article ID 101754, 12 pages
http://dx.doi.org/10.1155/2012/101754
Review Article

Clinical Trials and Treatment of ATL

1Department of Hematology, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Science, Nagasaki 852-8523, Japan
2Hematology and Hematopoietic Stem Cell Transplantation Division, National Cancer Center Hospital, Tokyo 104-0045, Japan

Received 1 August 2011; Accepted 29 September 2011

Academic Editor: Mineki Saito

Copyright © 2012 Kunihiro Tsukasaki and Kensei Tobinai. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

ATL is a distinct peripheral T-lymphocytic malignancy associated with human T-cell lymphotropic virus type I (HTLV-1). The diversity in clinical features and prognosis of patients with this disease has led to its subtype-classification into four categories, acute, lymphoma, chronic, and smoldering types, defined by organ involvement, and LDH and calcium values. In case of acute, lymphoma, or unfavorable chronic subtypes (aggressive ATL), intensive chemotherapy like the LSG15 regimen (VCAP-AMP-VECP) is usually recommended if outside of clinical trials, based on the results of a phase 3 trial. In case of favorable chronic or smoldering ATL (indolent ATL), watchful waiting until disease progression has been recommended, although the long-term prognosis was inferior to those of, for instance, chronic lymphoid leukemia. Retrospective analysis suggested that the combination of interferon alpha and zidovudine was apparently promising for the treatment of ATL, especially for types with leukemic manifestation. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is also promising for the treatment of aggressive ATL possibly reflecting graft versus ATL effect. Several new agent trials for ATL are ongoing and in preparation, including a defucosylated humanized anti-CC chemokine receptor 4 monoclonal antibody, IL2-fused with diphtheria toxin, histone deacetylase inhibitors, a purine nucleoside phosphorylase inhibitor, a proteasome inhibitor, and lenalidomide.