OBJECTIVES. Familial hemophagocytic lymphohistiocytosis (FHLH) is a genetically
determined disorder characterized by the early onset of fever, hepatosplenomegaly, central
nervous system disease, thrombocytopenia, coagulation disorders, and hemophagocytosis.
It is caused by genetic defects that impair T cell–mediated and natural cytotoxicity.
Chemotherapy-or immunotherapy-based treatments can achieve remission. Hematopoietic
stem cell transplantation (HSCT), however, is the only curative option, but optimal modalities …