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T-Cell Lymphomas in South America and Europe

Authors :
Monica Bellei
Carlos Sergio Chiattone
Stefano Luminari
Emanuela Anna Pesce
Maria Elena Cabrera
Carmino Antonio de Souza
Raul Gabús
Lucia Zoppegno
Jorge Milone
Astrid Pavlovsky
Joseph Michael Connors
Francine Mary Foss
Steven Michael Horwitz
Raymond Liang
Silvia Montoto
Stefano Aldo Pileri
Aaron Polliack
Julie Marie Vose
Pier Luigi Zinzani
Emanuele Zucca
Massimo Federico
Source :
Revista Brasileira de Hematologia e Hemoterapia, Vol 34, Iss 1, Pp 42-47 (2012)
Publication Year :
2012
Publisher :
Elsevier, 2012.

Abstract

Peripheral T-cell lymphomas are a group of rare neoplasms originating from clonal proliferation of mature post-thymic lymphocytes with different entities having specific biological characteristics and clinical features. As natural killer cells are closely related to T-cells, natural killer-cell lymphomas are also part of the group. The current World Health Organization classification recognizes four categories of T/natural killer-cell lymphomas with respect to their presentation: disseminated (leukemic), nodal, extranodal and cutaneous. Geographic variations in the distribution of these diseases are well documented: nodal subtypes are more frequent in Europe and North America, while extranodal forms, including natural killer-cell lymphomas, occur almost exclusively in Asia and South America. On the whole, T-cell lymphomas are more common in Asia than in western countries, usually affect adults, with a higher tendency in men, and, excluding a few subtypes, usually have an aggressive course and poor prognosis. Apart from anaplastic lymphoma kinase-positive anaplastic large cell lymphoma, that have a good outcome, other nodal and extranodal forms have a 5-year overall survival of about 30%. According to the principal prognostic indexes, the majority of patients are allocated to the unfavorable subset. In the past, the rarity of these diseases prevented progress in the understanding of their biology and improvements in the efficaciousness of therapy. Recently, international projects devoted to these diseases created networks promoting investigations on T-cell lymphomas. These projects are the basis of forthcoming cooperative, large scale trials to detail biologic characteristics of each sub-entity and to possibly individuate targets for new therapies.

Details

Language :
English
ISSN :
15168484 and 18060870
Volume :
34
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Revista Brasileira de Hematologia e Hemoterapia
Publication Type :
Academic Journal
Accession number :
edsdoj.b2663c39d43743a5825f50e421d3aa63
Document Type :
article
Full Text :
https://doi.org/10.5581/1516-8484.20120013