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Current Hematologic Malignancy Reports
Published in: Current Hematologic Malignancy Reports 6/2016

01-12-2016 | T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)

Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): An Update on Epidemiology, Clinical Presentation, and Natural History in North American and European Cases

Authors: Bradley M. Haverkos, Zenggang Pan, Alejandro A. Gru, Aharon G. Freud, Rachel Rabinovitch, Meng Xu-Welliver, Brad Otto, Carlos Barrionuevo, Robert A. Baiocchi, Rosemary Rochford, Pierluigi Porcu

Published in: Current Hematologic Malignancy Reports | Issue 6/2016

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Abstract

Purpose of Review

Extranodal NK/T cell lymphoma, nasal type (ENKTL-NT) is an aggressive extranodal non-Hodgkin lymphoma most commonly occurring in East Asia and Latin America but with increasing incidence in the United States. Data on epidemiology, disease presentation, and outcome for European and North American (“Western”) cases are very limited. We review published landmark clinical studies on ENKTL-NT in the West and report in detail recent data, including our institutional experience.

Recent Findings

We highlight key observations in its epidemiology, natural history, and trends in clinical management. In the USA, ENKTL-NT is more common among Asian Pacific Islanders (API) and Hispanics compared to non-Hispanic whites. Published studies indicate less heterogeneity in clinical presentation in Western ENKTL-NT compared to Asian patients. While there is variation in age at diagnosis, presence of antecedent lymphoproliferative disorders, and outcomes among racial/ethnic groups, the universal association of ENKTL-NT with EBV and the poor response of this neoplasm to anthracycline-based therapy is consistent across all geographic areas.

Summary

Data on epidemiology, disease presentation, and clinical outcomes in mature T cell and NK cell (T/NK cell) neoplasms, including ENKTL-NT, in Europe and North America are very limited. As the classification and diagnostic characterization of the currently recognized T/NK cell lymphoma disease entities continue to evolve, gaps and inconsistencies in data reporting across different studies are being recognized. Despite these limitations, several studies from the USA suggest that the incidence of ENKTL-NT is higher in Asian Pacific Islanders (API) and non-white Hispanics and that outcomes may be worse in non-whites. However, the universal association of ENKTL-NT with Epstein-Barr virus (EBV) across all ethnic groups suggests a common pathogenesis. Given the overlap between the entities included in the category of T/NK cell neoplasms, there is a need to further define biological and clinical differences that may affect diagnosis, treatment, and outcome.
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Metadata
Title
Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): An Update on Epidemiology, Clinical Presentation, and Natural History in North American and European Cases
Authors
Bradley M. Haverkos
Zenggang Pan
Alejandro A. Gru
Aharon G. Freud
Rachel Rabinovitch
Meng Xu-Welliver
Brad Otto
Carlos Barrionuevo
Robert A. Baiocchi
Rosemary Rochford
Pierluigi Porcu
Publication date
01-12-2016
Publisher
Springer US
Published in
Current Hematologic Malignancy Reports / Issue 6/2016
Print ISSN: 1558-8211
Electronic ISSN: 1558-822X
DOI
https://doi.org/10.1007/s11899-016-0355-9

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10-04-2024 Myocardial Infarction News

» View more highlights on the ACC 2024 congress hub page

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