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

  • T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)
  • Published:
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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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Authors and Affiliations

  1. Division of Hematology, University of Colorado, 1665 Aurora Ct., Mail Stop F754, Aurora, CO, 80045, USA

    Bradley M. Haverkos

  2. Department of Pathology, University of Colorado, Aurora, CO, USA

    Zenggang Pan

  3. Department of Pathology and Dermatology, University of Virginia, Charlottesville, VA, USA

    Alejandro A. Gru

  4. Department of Pathology, The Ohio State University, Columbus, OH, USA

    Aharon G. Freud

  5. Department of Radiology, University of Colorado, Aurora, CO, USA

    Rachel Rabinovitch

  6. Department of Radiation Oncology, The Ohio State University, Columbus, OH, USA

    Meng Xu-Welliver

  7. Department of Otolaryngology, Ohio State University, Columbus, OH, USA

    Brad Otto

  8. Department of Pathology, Instituto Nacional de Enfermedades Neoplasticas (I.N.E.N.), Lima, Peru

    Carlos Barrionuevo

  9. Division of Hematology and Comprehensive Cancer Center, Ohio State University, 320 West 10th Avenue, Columbus, OH, 43210, USA

    Robert A. Baiocchi & Pierluigi Porcu

  10. Department of Immunology and Microbiology, University of Colorado, Aurora, CO, USA

    Rosemary Rochford

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  2. Zenggang Pan
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  3. Alejandro A. Gru
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  4. Aharon G. Freud
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  11. Pierluigi Porcu
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Correspondence to Bradley M. Haverkos.

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Conflict of Interest

Bradley M. Haverkos, Zenggang Pan, Alejandro A. Gru, Aharon G. Freud, Rachel Rabinovitch, Meng Welliver, Brad Otto, Carlos Barrionuevo, Robert A. Baiocchi, and Rosemary Rochford each declared no potential conflicts of interest. Pierluigi Porcu is a section editor for Current Hematologic Malignancy Reports.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

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Haverkos, B.M., Pan, Z., Gru, A.A. et al. Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): An Update on Epidemiology, Clinical Presentation, and Natural History in North American and European Cases. Curr Hematol Malig Rep 11, 514–527 (2016). https://doi.org/10.1007/s11899-016-0355-9

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