Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

We report a case of a 5-year-old girl with EBV-associated hemophagocytic ly mphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transpl antation (CBSCT) from an unrelated donor. The patient presented with persis tent high-grade fever and hepatosplenomegaly, Because the disease was refra ctory to immunochemotherapy according to the HLH94 protocol, she received 2 .0 x 10(7) CB nucleated cells/kg body weight (BW) after conditioning with B U/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The n eutrophil count reached >0.5 x 10(9)/l by day 21 and the platelet count rea ched >50 x 10(9)/l by day 84. The patient recovered well with sequelae of n eurological deficits more than 10 months after receiving CBSCT, without sho wing evidence of HLH or chronic GVHD, Real-time PCR proved applicable for e stimation of the EBV load in PBMC of the patient. We conclude that CBSCT ma y be indicated for some cases of refractory EBV-HLH, who have no HLA-matche d siblings and are therefore dependent on unrelated marrow donors.