Bradley M Haverkos1, Zenggang Pan2, Alejandro A Gru3, Aharon G Freud4, Rachel Rabinovitch5, Meng Xu-Welliver6, Brad Otto7, Carlos Barrionuevo8, Robert A Baiocchi9, Rosemary Rochford10, Pierluigi Porcu9. 1. Division of Hematology, University of Colorado, 1665 Aurora Ct., Mail Stop F754, Aurora, CO, 80045, USA. bradley.haverkos@ucdenver.edu. 2. Department of Pathology, University of Colorado, Aurora, CO, USA. 3. Department of Pathology and Dermatology, University of Virginia, Charlottesville, VA, USA. 4. Department of Pathology, The Ohio State University, Columbus, OH, USA. 5. Department of Radiology, University of Colorado, Aurora, CO, USA. 6. Department of Radiation Oncology, The Ohio State University, Columbus, OH, USA. 7. Department of Otolaryngology, Ohio State University, Columbus, OH, USA. 8. Department of Pathology, Instituto Nacional de Enfermedades Neoplasticas (I.N.E.N.), Lima, Peru. 9. Division of Hematology and Comprehensive Cancer Center, Ohio State University, 320 West 10th Avenue, Columbus, OH, 43210, USA. 10. Department of Immunology and Microbiology, University of Colorado, Aurora, CO, USA.
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. 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.
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.
Entities:
Keywords:
ENKTL; Epstein–Barr virus; Europe; Extranodal NK/T cell lymphoma; United Kingdom; United States
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