Chronic active EBV infection
Chronic active EBV infection or in its expanded form, chronic active Epstein–Barr virus infection is a very rare and often fatal complication of Epstein–Barr virus infection that most often occurs in children or adolescents of Asian or South American lineage, although cases in Hispanics, Europeans and Africans have been reported. It is classified as one of the Epstein-Barr virus-associated lymphoproliferative diseases.
Presentation
The most common symptoms of CAEBV include:- Fatigue
- Fever
- Hepatitis
- Pancytopenia
- Spleen enlargement
- Hypersensitivity to mosquito bites
- Interstitial pneumonia
- Lymphoma, including B-cell, T-cell and NK-cell lymphomas
- Haemophagocytic syndrome
- Coronary artery aneurysms
- Liver failure
- Nasopharyngeal carcinoma
- Gastric adenocarcinoma
- Intestinal perforation
- Myocarditis
- Peripheral neuropathy
Pathophysiology
It arises from the cells that constitute the immune system, most often the T-cells and NK cells in Asians/South Americans and the B-cells in the other racial groups. Various cytokine anomalies have been reported in people with CAEBV, examples include:There is also evidence supporting a role for TGF-β in the disease. Those that develop the haemophagocytic syndrome often exhibit an abnormally high amount of IL-1β and IFN-γ.