Current Hematologic Malignancy Reports

, Volume 11, Issue 6, pp 514–527 | Cite as

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

  • 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
T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)
Part of the following topical collections:
  1. Topical Collection on T-Cell and Other Lymphoproliferative Malignancies


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.


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.


Extranodal NK/T cell lymphoma ENKTL Epstein–Barr virus United States Europe United Kingdom 


Compliance with Ethical Standards

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.

Human and Animal Rights and Informed Consent

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


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Bradley M. Haverkos
    • 1
  • Zenggang Pan
    • 2
  • Alejandro A. Gru
    • 3
  • Aharon G. Freud
    • 4
  • Rachel Rabinovitch
    • 5
  • Meng Xu-Welliver
    • 6
  • Brad Otto
    • 7
  • Carlos Barrionuevo
    • 8
  • Robert A. Baiocchi
    • 9
  • Rosemary Rochford
    • 10
  • Pierluigi Porcu
    • 9
  1. 1.Division of HematologyUniversity of ColoradoAuroraUSA
  2. 2.Department of PathologyUniversity of ColoradoAuroraUSA
  3. 3.Department of Pathology and DermatologyUniversity of VirginiaCharlottesvilleUSA
  4. 4.Department of PathologyThe Ohio State UniversityColumbusUSA
  5. 5.Department of RadiologyUniversity of ColoradoAuroraUSA
  6. 6.Department of Radiation OncologyThe Ohio State UniversityColumbusUSA
  7. 7.Department of OtolaryngologyOhio State UniversityColumbusUSA
  8. 8.Department of PathologyInstituto Nacional de Enfermedades Neoplasticas (I.N.E.N.)LimaPeru
  9. 9.Division of Hematology and Comprehensive Cancer CenterOhio State UniversityColumbusUSA
  10. 10.Department of Immunology and MicrobiologyUniversity of ColoradoAuroraUSA

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