Bader P, Klingebiel T, Klingel K, Föll J, Beck J, Handgretinger R, Ehninger G, Deeg H J, Niethammer D
Children's University Hospital, Dept. Hematology/Oncology, Tübingen, Germany.
Bone Marrow Transplant. 1995 Sep;16(3):479-81.
A 5-year-old boy with severe aplastic anemia failed to respond to cyclosporine (CYA), prednisolone and antilymphocyte globulin (ALG). No suitable sibling marrow donor was available, but an HLA-matched unrelated donor was identified. The patient was conditioned with cyclophosphamide (CY), 50 mg/kg/day for 4 days, total nodal irradiation (8 Gy), and ALG 30 mg/kg/day for 3 days. GVHD prophylaxis consisted of daily CYA, methotrexate (MTX) and ALG. The patient failed to achieve sustained engraftment. He was reconditioned with high-dose prednisolone and anti-T lymphocyte monoclonal antibody OKT3. The boy was reinfused with the same donor marrow on day 0 (+49/first BMT). No GVHD prophylaxis was given the second time. He received G-CSF on days 0 to +20 after the second transplant. Full engraftment was achieved on day +16 (+65). However, on day +31 (80) he developed a biopsy-proven B cell lymphoproliferative disorder (BLPD). After the OKT3 administration was stopped and treatment with ganciclovir and high-dose immunoglobulin was initiated, the BLPD resolved and the patient was discharged on day +50 (99). He is currently well with a functioning graft 266 (305) days posttransplant, with no sign of GVHD.
一名5岁的重度再生障碍性贫血男孩对环孢素（CYA）、泼尼松龙和抗淋巴细胞球蛋白（ALG）治疗无反应。没有合适的同胞骨髓供者，但找到了一名HLA匹配的无关供者。患者接受环磷酰胺（CY）预处理，50mg/kg/天，共4天，全身淋巴结照射（8Gy），以及ALG 30mg/kg/天，共3天。移植物抗宿主病（GVHD）预防措施包括每日使用CYA、甲氨蝶呤（MTX）和ALG。患者未能实现持续植入。他接受了大剂量泼尼松龙和抗T淋巴细胞单克隆抗体OKT3的再次预处理。该男孩在第0天（+49/首次骨髓移植）再次输注了同一供者的骨髓。第二次未采取GVHD预防措施。第二次移植后第0天至+20天他接受了粒细胞集落刺激因子（G-CSF）治疗。在第+16天（+65）实现了完全植入。然而，在第+31天（80），他经活检证实发生了B细胞淋巴增殖性疾病（BLPD）。在停用OKT3并开始使用更昔洛韦和大剂量免疫球蛋白治疗后，BLPD得到缓解，患者于第+50天（99）出院。目前，移植后266（305）天他情况良好，移植物功能正常，无GVHD迹象。
