Progress of allogeneic hematopoietic stem cell transplantation for hyper IgM syndrome / 国际儿科学杂志

ABSTRACT

Hyper immunoglobulin M syndrome(HIGM)is a group of rare primary immunodeficiency disease(PID)caused by single gene mutation.Allogeneic hematopoietic stem cell transplantation(allo-HSCT)is the only treatment to cure the disease, and patients should receive allo-HSCT if possible.Younger patients who can keep in good pre-transplant state, obtain matching sibling donor and tolerate to myeloablative conditioning may get better outcome after early transplantation, and the overall survival rate can reach 80%.The progress in the timing of transplantation, donor and graft, conditioning regimen, prevention and treatment of complications of allo-HSCT for HIGM is reviewed in this paper.