T-Cell Lymphomas in South America and Europe

Revista Brasileira de Hematologia e Hemoterapia

Monica Bellei²  Carlos Sergio Chiattone¹  Stefano Luminari²  Emanuela Anna Pesce²  Maria Elena Cabrera¹  Carmino Antonio De Souza¹  Raul Gabús¹  Lucia Zoppegno¹  Jorge Milone¹  Astrid Pavlovsky¹  Joseph Michael Connors¹  Francine Mary Foss¹  Steven Michael Horwitz¹  Raymond Liang¹  Silvia Montoto¹  Stefano Aldo Pileri¹  Aaron Polliack¹  Julie Marie Vose¹  Pier Luigi Zinzani¹  Emanuele Zucca¹  Massimo Federico² 

[1],L'Università di Modena e Reggio Emilia Department of Oncology, Hematology and Respiratory Diseases Modena,Italy

关键词: Lymphoma;    T-cell;    Killer-cells;    natural;    Prognosis;    Lymphoma;    T- cell;    Lymphoma;    T-Cell;    Hematologic neoplasms;    South America;    Europe;   

DOI  :  10.5581/1516-8484.20120013

来源: SciELO

【 摘 要 】

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.

【 授权许可】

CC BY-NC-ND   
 All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License