Contents
Severe progressive infection characterized by duration of > 6 months, persistent elevated EBV titers, and evidence of EBV-related organ damage.
- Characteristics:
- Severe progressive illness lasting > 6 months
- Lymphocytic infiltration of tissue (e.g., lymph nodes, lungs, liver, central nervous tissue, bone marrow, eye, and skin)
- ↑ EBV DNA & RNA in affected tissue
- Absence of any other immunosuppressive conditions
Classification
Chronic active EBV (CAEBV) type B cell:
CAEBV-type T/NK cell disease:
- Category A1: Polymorphic LPD with polyclonal proliferation of EBV-infected T/NK cells
- Category A2: Polymorphic LPD with monoclonal T/NK cells
- Category A3: Monomorphic LPD of monoclonal T cells
Clinical features
- Prolonged fever
- Hepatosplenomegaly
- Thrombocytopenia
- Anaemia
- Lymphadenopathy
Complications
Life-threatening complications:
- Hemophagocytic syndrome
- Interstitial pneumonia
- Malignant lymphoma
- Coronary aneurysms
- CNS involvement
Diagnosis
Histopathology

Lymph nodes:
- Paracortical hyperplasia with polymorphic and polyclonal lymphoid proliferation and large numbers of EBER positive cells
Liver:
- Portal/sinusoidal infiltration by small lymphocytes with no definite atypia