title: Prolonged resolution of hemophagocytic lymphohistiocytosis following myeloablative chemotherapy and subsequent autologous peripheral blood stem cell transplantation.
abstract: A 30-month-old boy with hemophagocytic lymphohistiocytosis (HLH) received an autologous peripheral blood stem cell transplant (PBSCT) following high-dose chemotherapy. He presented with hemophagocytic syndrome (HPS) at 6 months of age, but relapsed despite the repeated administration of prednisolone, VP-16, cyclosporin A (CsA), and other cytotoxic agents. PBSC were obtained using combination chemotherapy with etoposide (VP16, 450 mg/m2), doxorubicin (70 mg/m2), vincristine (2 mg/m2) and cyclophosphamide (CY, 1200 mg/m2). 2.7 x 10(5)/kg CFU-GM PBSC were transplanted after similar high-dose VP16 preconditioning used for allogeneic BMT for HLH. The boy continues to remain in complete remission 30 months after PBSCT while receiving low-dose PSL/CsA therapy. High-dose chemotherapy followed by PBSCT may be an optional therapeutic approach for patients with HLH.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Ohga
authors.forename: A
authors.initials: A
authors.lastname: Nomura
authors.forename: T
authors.initials: T
authors.lastname: Kai
authors.forename: A
authors.initials: A
authors.lastname: Matsuzaki
authors.forename: S
authors.initials: S
authors.lastname: Inaba
authors.forename: M
authors.initials: M
authors.lastname: Suda
authors.forename: K
authors.initials: K
authors.lastname: Ueda
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D018380:Hematopoietic Stem Cell Transplantation; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D019172:Transplantation Conditioning; D014182:Transplantation, Autologous