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)
Classification
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
cysts)
- 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
- 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