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镰状细胞贫血(SCA)仍是一种发病率和死亡率很高的疾病。治疗设计旨在预防血管闭塞发作和感染。一旦发生这些情况,主要是症状性治疗。1986年4月以来,作者对5例SCA施行了同种异体骨髓移植(BMT),初步结果报告如下。患者供体和方法 5例严重SCA患儿均为扎伊尔黑种人,男1例,女4例;年龄为11个月～91/2岁。患儿每年血管闭塞发作多达4次,且每年需4次输血。BMT时均无慢性器官损害。以前的治疗包括叶酸和吡烷酮醋胺。血红蛋白均为SS型。供体为与患者同种组织相容性抗原(HLA)相同(4名同胞,1名父亲),血红蛋白电泳或正常(2名AA)或为镰状细胞性状携带者(3名AS)。 BMT接受者准备包括用白消安(4 mg/kg/d)4天,然后再用环磷酰胺(50mg/kg/d)4天。为
Sickle-cell anemia (SCA) remains a disease with a high morbidity and mortality. Treatment designed to prevent the onset of vascular occlusion and infection. In the event of these conditions, the main is symptomatic treatment. Since April 1986, the authors have performed allogeneic bone marrow transplantation (BMT) on 5 patients with SCA. The preliminary results are reported below. Patient Donors and Methods Five children with severe SCA were all Zaire blacks, including 1 males and 4 females, ranging in age from 11 months to 91 years and 2 years. Children with vascular occlusion episodes up to 4 times per year, and 4 times a year to be transfused. No chronic organ damage at BMT. Previous treatments include folic acid and pyrrolidone amine. Hemoglobin is SS type. The donor was identical to the patient’s HLA (4 siblings, 1 father), hemoglobin electrophoresis or normal (2 AA) or sickle cell trait (3 AS). BMT recipients were included for 4 days with busulfan (4 mg / kg / d) and then cyclophosphamide (50 mg / kg / d) for 4 days. for