Ohga S, Nomura A, Kai T, Matsuzaki A, Inaba S, Suda M, Ueda K
Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Bone Marrow Transplant. 1997 Mar;19(6):633-5. doi: 10.1038/sj.bmt.1700702.
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.
一名30个月大的噬血细胞性淋巴组织细胞增生症（HLH）男孩在接受大剂量化疗后接受了自体外周血干细胞移植（PBSCT）。他在6个月大时出现噬血细胞综合征（HPS），尽管反复使用泼尼松龙、依托泊苷（VP-16）、环孢素A（CsA）和其他细胞毒性药物，但仍复发。使用依托泊苷（VP16，450mg/m²）、阿霉素（70mg/m²）、长春新碱（2mg/m²）和环磷酰胺（CY，1200mg/m²）联合化疗获取外周血干细胞。在用于HLH异基因骨髓移植（BMT）的类似大剂量VP16预处理后，移植了2.7×10⁵/kg集落形成单位粒细胞-巨噬细胞（CFU-GM）外周血干细胞。该男孩在接受低剂量泼尼松龙/环孢素A治疗的情况下，PBSCT后30个月仍持续完全缓解。大剂量化疗后进行PBSCT可能是HLH患者的一种可选治疗方法。
