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21 - Peripheral T-cell lymphomas

Published online by Cambridge University Press:  10 January 2011

By
Matthew J. Matasar  and
Steven M. Horwitz
Edited by
Susan O'Brien ,
Julie M. Vose  and
Hagop M. Kantarjian
Matthew J. Matasar
Affiliation:
Department of Medicine, Lymphoma Service and Hematology Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Steven M. Horwitz
Affiliation:
Department of Medicine, Lymphoma Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Susan O'Brien
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
Julie M. Vose
Affiliation:
University of Nebraska Medical Center, Omaha
Hagop M. Kantarjian
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
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Summary

Introduction

The T-cell non-Hodgkin lymphomas are a group of uncommon malignancies that in Western countries account for 15–20% of aggressive lymphomas and between 5% and 10% of all non-Hodgkin's lymphomas (NHL). Within the most current classification schemata, there are 21 distinct diseases that together constitute the mature T- and natural killer (NK)-cell lymphomas, diseases that range from indolent cutaneous lymphomas to aggressive malignancies that are often resistant to routine systemic therapy. Common to many of the types of T-cell lymphoma is a predilection towards extranodal disease, although sites of tropism vary among subtypes. Geographic frequencies of many subtypes, and of T-cell lymphomas as a group, are also highly variable: while only 1.5% of lymphomas are of T-cell lineage in Vancouver, Canada, 18.3% of lymphomas in Hong Kong show a T-cell phenotype. And in Asia, 47.4% of T-cell lymphomas are either NK/T-cell lymphoma, nasal type (NK/T-NT) or adult T-cell leukemia/lymphoma (ATLL), while in North America and Europe, these constitute only 7.1% and 5.3% of T-cell lymphomas, respectively. These variable incidence rates may in part represent differential exposure to risk factors for T-cell lymphoma, including Epstein–Barr virus (EBV) and human T-cell leukemia virus-1 (HTLV-1). As will be explored below, the epidemiologic variety across the subtypes of T-cell lymphoma is matched by the clinical heterogeneity of these diseases, confounding investigation (or understanding) of the class of diseases as a single entity.

Type
Chapter
Information
Management of Hematologic Malignancies , pp. 410 - 431
Publisher: Cambridge University Press
Print publication year: 2010

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