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Clinical manifestations, pathologic features, and diagnosis of adult T cell leukemia-lymphoma

Authors
Arnold S Freedman, MD
Jon C Aster, MD
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Adult T cell leukemia-lymphoma (ATL), according to the most recent World Health Organization (WHO) classification of lymphoid neoplasms, is defined as a peripheral T cell neoplasm associated with infection by the human T-lymphotropic virus, type I (HTLV-I) [1,2]. Although it is considered one of the highly aggressive T cell non-Hodgkin lymphoma (NHL) variants, the disease course is variable and sometimes quite indolent. (See "Classification of the hematopoietic neoplasms".)

Other distinct T cell neoplasms include mycosis fungoides (cutaneous T cell lymphoma), T cell large granular lymphocytic leukemia, T cell prolymphocytic leukemia, anaplastic large cell lymphoma, peripheral T cell lymphoma, and precursor T cell lymphoblastic leukemia/lymphoma. These disorders are not caused by HTLV-1.

Epidemiologic studies suggest that HTLV-I is primarily transmitted by breastfeeding, although spread via blood transfusion, sharing of needles, and sexual intercourse also occurs. This is discussed in more detail separately. (See "Human T-lymphotropic virus type I: Virology, pathogenesis, and epidemiology".)

The epidemiology, pathogenesis, clinical features, pathology, and diagnosis of ATL will be discussed here; the treatment of ATL is discussed separately. (See "Treatment and prognosis of adult T cell leukemia-lymphoma".)

EPIDEMIOLOGY

Adult T cell leukemia-lymphoma (ATL) is an uncommon lymphoid neoplasm that occurs in patients with human T-lymphotropic virus, type I (HTLV-I) infection. Incidence varies by population according to the prevalence of HTLV-I infection [3]. Infection with HTLV-1 is endemic in several islands in southern Japan, the Caribbean basin (eg, Jamaica and Trinidad), western Africa, Peru, northeast Iran, and the southeastern portion of the United States; most affected patients live in or originate from these areas [4-10].

                         

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Literature review current through: Nov 2016. | This topic last updated: Tue Mar 29 00:00:00 GMT 2016.
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