[Rinsho ketsueki] The Japanese journal of clinical hematology | 2019
[Hematopoietic cell transplantation for chronic active EBV infection].
Abstract
Chronic active EBV infection (CAEBV) is one of the EBV-associated T/NK lymphoproliferative diseases. The prognosis of CAEBV is very poor, and without curative therapy, almost half of patients will die within five years of onset. The results of a 3-step treatment regimen consisting of step 1 (immunochemotherapy), step 2 (multi-drug chemotherapy), and step 3 (hematopoietic cell transplantation, HCT) are excellent. HCT is the only cure for CAEBV, and reduced-intensity conditioning (RIC) is superior to myeloablative conditioning (MAC). The overall survival rate is typically more than 90% following bone marrow transplantation and cord blood transplantation.