EBV-associated T and NK cell lymphoproliferative disorders: Consensus report of the 4th Asian Hematopathology Workshop

Young Hyeh Ko, Hyun Jung Kim, Young Ha Oh, Geongsin Park, Seung Sook Lee, Jooryung Huh, Chul Woo Kim, Insun Kim, Siok Bian Ng, Soo Young Tan, Shih Sung Chuang, Naoya Nakamura, Tadashi Yoshino, Shigeo Nakamura, Hiroshi Kimura, Koichi Ohshima

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12 Citations (Scopus)


T and NK cell proliferative diseases associated with acute and chronic Epstein-Barr virus (EBV) infection are uncommon and raise several clinical issues regarding diagnostic criteria and terminology. This is a summary report of the consensus meeting held in the 4th Asian Hematopathology Workshop. The umbrella term "EBV-positive T/NK lymphoproliferative disease in childhood-type" covers the entire spectrum of EBV-associated lesions in childhood, ranging from reactive to neoplastic processes. Systemic T/NK cell lymphoproliferative disease (LPD) of childhood type is defined as a fulminant disease associated with the proliferation of polyclonal, oligoclonal, or monoclonal T or NK cells and includes aggressive NK cell-associated leukemia in children. Chronic active EBV disease-type T/NK cell LPD is divided into three groups-polymorphic/polyclonal, polymorphic/monoclonal, and monomorphic/monoclonal-based on the histology and clonality of T or NK cells. A monoclonal EBV-positive T/NK cell type of proliferation with the clinical features of chronic active EBV disease rather than the fulminant course of systemic EBV-positive T/NK cell LPD of childhood is considered "chronic active EBV disease-type T/NK cell LPD". Hydroa vacciniforme (HV)-like T cell LPD is defined as a spectrum of EBV-infected T cell proliferative diseases with homing to the skin and is further classified into a classic type, a severe type, and malignant lymphoma. Criteria to define each category of HV-like T cell LPD remain to be clarified.

Original languageEnglish
Pages (from-to)319-324
Number of pages6
JournalJournal of Hematopathology
Issue number4
Publication statusPublished - Dec 2012


  • Chronic active infection
  • EBV
  • Hemophagocytosis
  • Hydroa vacciniforme
  • Lymphoproliferative disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Hematology


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