2014 Volume 3 Issue 1 Pages 1-11
Just a half century has passed since the discovery of Epstein-Barr virus (EBV). Chronic active EBV infection (CAEBV) is a prototype of EBV-associated T- or NK-cell lymphoproliferative diseases (EBV+T/NK-cell LPDs). The latest guideline for diagnosing CAEBV was proposed by Okano et al (2005). Definitive diagnosis requires evidence of EBV-infected T/NKcell proliferation (practically, evidence of high EBV load and EBV infection to T/NK cells) in the patient. However, the necessary tests are not covered by National Health Insurance, so a more invasive approach (a biopsy of the involved tissue) is needed. Because CAEBV patients possibly experience lethal hypercytokinemia, they should undergo immunochemotherapy followed by multi-drug chemotherapy, and need a preparation of allogeneic hematopoietic stem celltransplantation (HSCT). In cases of insufficient control of the symptoms and a progression of the disease, an allogeneic HSCT might be urgently required. Reduced-intensity conditioning is better than myeloablative conditioning, the overall survival rate is equally > 90% after bone marrow transplantation and cord blood transplantation, and the outcome is not inferior in young adults (<40 years old) compared to that in children.
