[Update on allogeneic hematopoietic stem cell transplantation in chronic active Epstein-Barr virus disease]

Chronic active Epstein-Barr virus (EBV) disease (CAEBV) is the prototype of EBV-associated T/NK-cell lymphoproliferative diseases (LPDs). CAEBV is a chronic/progressive LPD with a high mortality rate if left untreated with only symptomatic measures. The basic treatment consists of three steps: (1) cooling with steroids, (2) multi-drug combination chemotherapy, and (3) allogeneic hematopoietic stem cell transplantation (HSCT). Once CAEBV is diagnosed, the safest approach is to begin an initial treatment as soon as possible and complete the final treatment while there is still time. New drugs and approaches for each of the 3 steps have emerged in recent years, but HSCT remains the only curative treatment. Many retrospective analyses of HSCT have been published, and have contributed to progress in optimizing transplantation.