29. Therapeutic Approaches to the Mature T-Cell Lymphoproliferative Leukemias

  1. Stefan Faderl MD Associate Professor and
  2. Hagop Kantarjian MD Chairman Professor
  1. Nnenna Osuji Honorary Consultant1 and
  2. Claire Dearden MD, FRCP, FRCPATH Consultant Haematologist2

Published Online: 4 JAN 2011

DOI: 10.1002/9781444327359.ch29

Leukemias: Principles and Practice of Therapy

Leukemias: Principles and Practice of Therapy

How to Cite

Osuji, N. and Dearden, C. (2010) Therapeutic Approaches to the Mature T-Cell Lymphoproliferative Leukemias, in Leukemias: Principles and Practice of Therapy (eds S. Faderl and H. Kantarjian), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444327359.ch29

Editor Information

  1. Department of Leukemia, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

Author Information

  1. 1

    Section of Haemato-Oncology, Royal Mardsen NHS Foundation Trust/Institute of Cancer, Research, Surrey, UK

  2. 2

    Royal Marsden NHS Foundation Trust, Surrey, UK

Publication History

  1. Published Online: 4 JAN 2011
  2. Published Print: 26 NOV 2010

ISBN Information

Print ISBN: 9781405182355

Online ISBN: 9781444327359

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Keywords:

  • T-cell;
  • prolymphocytic leukemia;
  • large granular lymphocytes;
  • NK cell;
  • monoclonal antibodies

Summary

Management of the heterogeneous group of T/NK-cell lymphoproliferative disorders [T-cell prolymphocytic leukemia (T-PLL), T-cell large granular lymphocyte leukemia (T-cell LGL), adult T-cell leukemia/lymphoma (ATLL), and aggressive NK-cell leukemia] remains challenging. The rarity of these conditions, their refractoriness to standard therapies, underlying immune suppression, multi-factorial etiologies and lack of single identifiable therapeutic targets in the majority of cases, have all contributed to this status quo. There are emerging data from case series, phase II studies, and a small number of randomized trials. For T-PLL, alemtuzumab is now accepted as the standard first-line treatment. In other disorders such as T-cell LGL, control of cytopenias using immunosuppressants, rather than attempts to eradicate the malignant clone with cytotoxics, is recommended. In ATLL, the addition of antiviral therapies has improved outcome. Other considerations include supportive therapies, maintenance and consolidation approaches, particularly allogeneic transplantation, and targeting of underlying pathogenic mechanisms. Collaborative research is needed to optimize treatment strategies for these disorders.