Chronic active EBV infection
| Chronic active EBV infection | |
|---|---|
| Other names | CAEBV |
haematopoietic stem cell transplant[1] | |
Chronic active EBV infection or in its expanded form, chronic active Epstein–Barr virus infection is a very rare and often fatal complication of
EBV+ LPD).[2]
Presentation
The most common symptoms of CAEBV include:[1][3][4][5]
- Fever
- Hepatitis
- Pancytopenia
- Spleen enlargement
- Hypersensitivity to mosquito bites
Complications include:[1][3][5]
- Interstitial pneumonia
- Lymphoma, including B-cell, T-cell and NK-cell lymphomas[6]
- Haemophagocytic syndrome
- Coronary artery aneurysms
- Liver failure
- Nasopharyngeal carcinoma
- Gastric adenocarcinoma
- CNS
- Intestinal perforation
- Myocarditis
- Peripheral neuropathy
Pathophysiology
It arises from the cells that constitute the immune system, most often the
B-cells in the other racial groups.[1] Various cytokine anomalies have been reported in people with CAEBV, examples include:[5][7]
There is also evidence supporting a role for
haemophagocytic syndrome often exhibit an abnormally high amount of IL-1β and IFN-γ.[8]
Diagnosis
![[icon]](/File:Wiki_letter_w_cropped.svg){kind=small} | This section is empty. You can help by adding to it. (February 2019) |
Treatment
The only known cure for CAEBV is allogenic
haematopoietic stem cell transplant (HSCT), with all other treatment options (rituximab, cytotoxic chemotherapy and immunosuppressive therapy) being nothing more than stopgaps.[1][3][5][7]
Prognosis
Without HSCT the condition is inevitably fatal and even HSCT is no guarantee, with a significant portion of patients dying from the disease progression.[8] Factors indicative of a poor prognosis include: thrombocytopenia, late onset of the disease (age ≥ 8 years) and T cell involvement.[9]