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Table 1 Natural killer cell neoplasms according to the World Health Organizationclassification of tumors of hematopoietic and lymphoid tissues

From: Aggressive mature natural killer cell neoplasms: from epidemiology todiagnosis

WHO classification, 2008 Disease entities Putative cells of origin Orphanet numbers Synonyms in the Orphanet data base ICD codes Prevalence categories (/1.000.000) [References]
      ICD-10 ICD-O-3   
NK-cell neoplasms Extranodal NK/T-cell lymphoma (NKTCL), nasal type Mature NK-cells 86879 NK/T-cell lymphoma; Nasal T/natural killer-cell lymphoma;Angiocentric T-cell lymphoma; Lethal midline granuloma C86.0 9719/3 <1-9 [19, 2224]
Aggressive NK-cell leukemia (ANKCL) Mature NK-cells 86873 Aggressive NK-cell leukemia; Aggressive NK-cell lymphoma; NK-cellLGL leukemia; NK-cell large granular lymphocyte leukemia C94.7 9948/3 <1-9 [20, 2224]
Provisional entities Chronic lymphoproliferative disorders of NK-cells (CLPD-NK) Mature NK-cells Not available NK-cell LGL leukemia; NK-cell large granular lymphocyte leukemia(considered together with ANKCL) Not available (C94.7)* Not available (9831/3)** Unknown [2124]
  NK-cell lymphoblastic leukemia / lymphoma NK-, T- and/or myeloid precursor cells Not available Not available Not available Not available Unknown [2225]
  1. Abbreviations: ICD-10 International Statistical Classificationof Diseases and Related Health Problems (formerly designatedInternational Classification for Diseases), 10th revision, 2010, WorldHealth Organization (available in:;accessed in 2 February 2013); ICD-O-3 International StatisticalClassification of Diseases and Related Health Problems, for Oncology(formerly designated International Classification of Diseases forOncology, 3rd edition, 2000, World Health Organization (available in:;accessed in 2 February 2013); WHO World HealthOrganization.
  2. Blastic plasmacytoid dendritic cell neoplasm, also known asCD4+ CD56+ hematodermic neoplasm andpreviously designed blastic NK-cell lymphoma (Orpha:86870; ICD-10:C86.4, still referred as blastic NK-cell lymphoma; ICD-O: 9727/3) arisesfrom plasmacytoid dendritic cells and should no longer be considered aNK-cell malignancy [26].
  3. * Considering the synonymous list, CLPD-NK, which include chronic NK-cellLGL leukemia cases, are considered together with ANKCL; ** According tothe proposal of the WHO classification, 2008, CLPD-NK should beconsidered together with T-LGLL in the ICD-O.