Chronic active EBV infection

Chronic active Epstein-Barr virus infection is a very rare and often fatal illness in which the body fails to control an Epstein-Barr virus infection for at least six months after the first infection. It most often affects children and adolescents of Asian or South American lineage, though cases have been reported in Hispanic, European and African people. It is classed among the Epstein-Barr virus-associated lymphoproliferative diseases. The disease arises from cells of the immune system, usually T cells and NK cells in Asian and South American patients and B cells in other groups, and unusual cytokine levels have been reported. The only known cure is allogeneic haematopoietic stem cell transplant, while rituximab, cytotoxic chemotherapy and immunosuppressive therapy only hold the disease back. Without a transplant the condition is described as inevitably fatal, and a poor outlook is linked to low platelet counts, onset at age eight or older and T cell involvement.