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Establishment of Epstein-Barr Virus Growth-transformed Lymphoblastoid Cell Lines
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Cutaneous EBV-related lymphoproliferative disorders.

Alejandro A Gru1, Elaine S Jaffe2

  • 1Pathology & Dermatology, Hematopathology and Dermatopathology Sections, University of Virginia, Charlottesville, VA, USA.

Seminars in Diagnostic Pathology
|December 19, 2016
PubMed
Summary

This article discusses Epstein-Barr virus (EBV)-associated cutaneous lymphoproliferative disorders and upcoming WHO classification changes. It highlights T/NK-cell and B-cell EBV+ LPDs, including new classifications and their prevalence in specific populations.

Keywords:
Chronic active EBV infectionEBV+ mucocutaneous ulcerExtranodal NK/T-cell lymphomaHydroa vacciniforme

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Area of Science:

  • Hematology
  • Oncology
  • Dermatology

Background:

  • Cutaneous lymphoproliferative disorders (LPD) are associated with Epstein-Barr virus (EBV).
  • The 4th Edition of the WHO Classification of Tumors of the Hematopoietic and Lymphoid Tissues introduces significant changes, particularly for EBV-associated T- and NK-cell neoplasms.
  • Extranodal NK/T-cell lymphoma, primarily of the upper aerodigestive tract, can secondarily involve the skin.

Purpose of the Study:

  • To review EBV-associated cutaneous lymphoproliferative disorders.
  • To emphasize the forthcoming revisions in the WHO classification concerning these entities.
  • To detail specific T/NK-cell and B-cell EBV-associated LPDs.

Main Methods:

  • Review of current literature and classification guidelines.
  • Focus on changes within the WHO 4th Edition for hematopoietic and lymphoid tumors.
  • Categorization of EBV-associated LPDs based on cell lineage (T/NK-cell vs. B-cell).

Main Results:

  • EBV-associated T/NK-cell LPDs include systemic chronic active EBV infection (CAEBV), systemic EBV+ T-cell lymphoma of childhood, and hydroa vacciniforme (HV)-like LPD.
  • HV-like LPD is a cutaneous manifestation of CAEBV.
  • These T/NK-cell disorders are more prevalent in Asian and indigenous populations of the Americas.
  • WHO classification changes for B-cell EBV+ LPDs include renaming EBV-positive diffuse large B-cell lymphoma (EBV-DLBCL) of the elderly to EBV-DLBCL, not otherwise specified (NOS).
  • A new entity, EBV+ mucocutaneous ulcer, involving skin and mucosal sites, has been added.

Conclusions:

  • The revised WHO classification brings important updates to EBV-associated cutaneous lymphoproliferative disorders.
  • Understanding these changes is crucial for accurate diagnosis and management.
  • EBV plays a significant role in various cutaneous lymphoid neoplasms across different cell types.