Ho C M, Vowels M R, Lockwood L, Ziegler J B
Department of Haematology/Oncology, Prince of Wales Children's Hospital, Sydney, Australia.
Bone Marrow Transplant. 1996 Jul;18(1):213-5.
A 16-month-old Aboriginal boy was diagnosed with chronic granulomatous disease (CGD) when he presented with frequent significant infections from the age of 12 months. BMT was performed from a HLA-matched sibling after conditioning with busulphan, cyclophosphamide, etoposide and antithymocyte globulin. A small bowel obstruction developed in the first week and settled with conservative treatment. VOD occurred in the third week, resolving after treatment with tissue plasminogen activator. Sustained engraftment has occurred, as indicated by return of the nitroblue tetrazolium (NBT) test to normal when performed at 11 weeks and 7 months post-BMT. No further infections have occurred. BMT can be successfully performed for CGD. Complications occurring post-BMT may be related to the underlying disease (CGD). BMT remains an attractive option for CGD in children who have a matched sibling donor.
一名16个月大的原住民男孩，从12个月大起就频繁出现严重感染，被诊断为慢性肉芽肿病（CGD）。在接受白消安、环磷酰胺、依托泊苷和抗胸腺细胞球蛋白预处理后，由一名HLA匹配的同胞进行了骨髓移植（BMT）。第一周出现小肠梗阻，经保守治疗后缓解。第三周发生肝静脉闭塞病（VOD），经组织纤溶酶原激活剂治疗后消退。如在BMT后11周和7个月进行的硝基蓝四唑（NBT）试验恢复正常所示，已实现持续植入。此后未再发生感染。CGD患者可成功进行BMT。BMT后出现的并发症可能与基础疾病（CGD）有关。对于有匹配同胞供体的儿童CGD患者，BMT仍然是一个有吸引力的选择。
