Abstract
Hemophagocytic syndrome/hemophagocytic lymphohistiocytosis (HPS/HLH) is a hypercytokinemic syndrome which is characterized by fever, hepatosplenomegaly, disseminated intravascular coagulopathy and bone marrow hemophagocytosis. Half of the patients with primary HLH carry the mutation of responsible genes associated with cytotoxic granules governing the immune homeostasis, which explains the rationale for allogeneic hematopoietic stem cell transplantation (HSCT) as the sole curative treatment. On the other hand, there remain many problems concerning the appropriate time and method of HSCT since HLH infants have considerable transplantation-related mortality. Reemerging HLH and central nervous system disease during the course of HSCT are the challenge inherent in the disease. The treatment modality should be individualized for patients with atypical presentation including adult onset HLH or lymphoma. Because of the small number of refractory cases with Epstein-Barr virus associated HLH that require allogeneic HSCT, the application and method of transplantation have not been established. We herewith review the recent advances and pressing problems concerning HSCT in patients with primary HLH or EBV-HLH.
