Allogeneic hematopoietic stem cell transplantation for childhood hemophagocytic lymphohistiocytosis / 中华实用儿科临床杂志

ABSTRACT

Childhood hemophagocytic lymphohistiocytosis (HLH) can be classified into primary and secondary forms.With the progress of diagnosis and treatment of stratified treatment, the prognosis was improved significantly.Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is recommended for the treatment of primary HLH, recurrent/refractory HLH and central nervous system involved HLH.With the development of transplantation technology, transplantation related mortality (TRM) has decreased significantly.In general, remission period of HLH is the best time for transplantation, while in some special situation HSCT can be done when HLH is still active.In addition to HLA matched sibling donors, the survival rates of unrelated matched donors and haploidentical donors were significantly improved.Most of the domestic institution choose the improved myeloablative conditioning as the conditioning regimen.