Internal Medicine

EBV-associated lymphoproliferative diseases (EBV+LPD)

Group of disorders in which one or more types of lymphoid cells, i.e. B cells, T cells, NK cells, and histiocytic-dendritic cells, are infected with the Epstein-Barr virus (EBV), proliferate excessively, and are associated with the development of various non-malignant, pre-malignant, and malignant lymphoproliferative disorders (LPD).

  • Oncogenic potential of EBV is reflected in its ability to infect and transform B lymphocytes into continously growing lymphoblastoid cell lines (LCL) (Pope et al. 1968)


EBV associated B-cell lymphoproliferative diseases:

  • Infectious mononucleosis
  • Chronic active EBV (CAEBV) of B-cell type
    • Infection characterized by a severe illness > 6 months, persistent elevated EBV titers, and evidence of EBV-related organ damage
  • EBV–positive diffuse large B-cell lymphoma (DLBCL)
  • EBV mucocutaneous ulcer
    • Characterized by the presence of mucocutaneous ulcers with an indolent clinical course
  • DLBCL associated with chronic inflammation
    • Inflammation develops in the setting of long-standing chronic inflammation with EBV association usually involving body cavities or enclosed spaces (like
  • Lymphomatoid granulomatosis

EBV-associated T-cell and NK cell lymphoproliferative diseases:

  • EBV-associated hyperinflammatory syndrome:
    • EBV-associated hemophagocytic lymphohistiocytosis
  • CAEBV-type T/NK cell disease:
    • Systemic chronic active EBV infection of T/NK cell type
    • Cutaneous forms of CAEBV:
      • Severe mosquito bite allergy
      • Hydroa vacciniforme-like lymphoproliferative disease
  • Malignant T/NK cell disease:
    • Systemic EBV-positive T-cell lymphoma
    • Extranodal NK/T cell lymphoma, nasal type
      • Characterized by frequent necrosis, angiocentric growth, cytotoxic phenotype and strong EBV association
    • Extranasal NK/T cell lymphoma
      • Involve the skin, GI tract, testis, and soft tissue
    • Aggressive NK cell leukemia
      • Primarily involves peripheral blood and bone marrow
    • EBV-positive nodal NK/T cell lymphoma (provisional)
      • Involve limited number of extranodal organs except for the nasal cavity, but the main bulk located in lymph nodes


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