Journal of Hematopathology

, Volume 5, Issue 4, pp 319–324 | Cite as

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
Review Article


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.


EBV Lymphoproliferative disease Chronic active infection Hemophagocytosis Hydroa vacciniforme 


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Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Young-Hyeh Ko
    • 1
  • Hyun-Jung Kim
    • 2
  • Young-Ha Oh
    • 3
  • Geongsin Park
    • 4
  • Seung-Sook Lee
    • 5
  • Jooryung Huh
    • 6
  • Chul-Woo Kim
    • 7
  • Insun Kim
    • 8
  • Siok-Bian Ng
    • 9
  • Soo-Young Tan
    • 10
  • Shih-Sung Chuang
    • 11
  • Naoya Nakamura
    • 12
  • Tadashi Yoshino
    • 13
  • Shigeo Nakamura
    • 14
  • Hiroshi Kimura
    • 15
  • Koichi Ohshima
    • 16
  1. 1.Department of Pathology, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  2. 2.Department of PathologyInje University, Sanggye Paik HospitalSeoulSouth Korea
  3. 3.Department of PathologyHanyang University Guri HospitalGuri-siSouth Korea
  4. 4.Catholic University St. Mary’s HospitalSeoulSouth Korea
  5. 5.Department of Pathology, Korea Cancer Center HospitalKorea Institute of Radiological and Medical SciencesSeoulSouth Korea
  6. 6.Department of Pathology, Asan Medical CenterUniversity of Ulsan College of MedicineSeoulSouth Korea
  7. 7.Department of PathologySeoul National University HospitalSeoulSouth Korea
  8. 8.Department of PathologyKorea University College of MedicineSeoulSouth Korea
  9. 9.Department of PathologyNational University Health SystemSingaporeSingapore
  10. 10.Department of PathologySingapore General HospitalSingaporeSingapore
  11. 11.Department of PathologyChi-Mei Medical CentreTainanTaiwan
  12. 12.Department of PathologyTokai University School of MedicineIseharaJapan
  13. 13.Department of PathologyOkayama University Graduate School of MedicineOkayamaJapan
  14. 14.Department of PathologyNagoya UniversityNagoyaJapan
  15. 15.Department of VirologyNagoya UniversityNagoyaJapan
  16. 16.Department of Pathology, School of MedicineKurume UniversityKurumeJapan

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