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.
abstract: 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.
  
  
  
  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.
authors: Division of Hematology, University of Colorado, 1665 Aurora Ct., Mail Stop F754, Aurora, CO, 80045, USA. bradley.haverkos@ucdenver.edu.
authors: Bradley M
authors: BM
authors: Haverkos
authors: Department of Pathology, University of Colorado, Aurora, CO, USA.
authors: Zenggang
authors: Z
authors: Pan
authors: Department of Pathology and Dermatology, University of Virginia, Charlottesville, VA, USA.
authors: Alejandro A
authors: AA
authors: Gru
authors: Department of Pathology, The Ohio State University, Columbus, OH, USA.
authors: Aharon G
authors: AG
authors: Freud
authors: Department of Radiology, University of Colorado, Aurora, CO, USA.
authors: Rachel
authors: R
authors: Rabinovitch
authors: Department of Radiation Oncology, The Ohio State University, Columbus, OH, USA.
authors: Meng
authors: M
authors: Xu-Welliver
authors: Department of Otolaryngology, Ohio State University, Columbus, OH, USA.
authors: Brad
authors: B
authors: Otto
authors: Department of Pathology, Instituto Nacional de Enfermedades Neoplasticas (I.N.E.N.), Lima, Peru.
authors: Carlos
authors: C
authors: Barrionuevo
authors: Division of Hematology and Comprehensive Cancer Center, Ohio State University, 320 West 10th Avenue, Columbus, OH, 43210, USA.
authors: Robert A
authors: RA
authors: Baiocchi
authors: Department of Immunology and Microbiology, University of Colorado, Aurora, CO, USA.
authors: Rosemary
authors: R
authors: Rochford
authors: Division of Hematology and Comprehensive Cancer Center, Ohio State University, 320 West 10th Avenue, Columbus, OH, 43210, USA.
authors: Pierluigi
authors: P
authors: Porcu
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001215:Asparaginase / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D004279:DNA, Viral / Q000032:analysis; D005060:Europe / Q000453:epidemiology; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification / Q000502:physiology; D006801:Humans; D054391:Lymphoma, Extranodal NK-T-Cell / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology* / Q000821:virology; D009656:North America / Q000453:epidemiology
chemical_list: D000970:Antineoplastic Agents; D015415:Biomarkers; D004279:DNA, Viral; D001215:Asparaginase
keywords: ENKTL; Epstein–Barr virus; Europe; Extranodal NK/T cell lymphoma; United Kingdom; United States