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骨髓移植(BMT)使得年轻病人的镰状细胞病得以治愈,然而,作为规定方案的一部分,所给予的马利兰和环磷酰胺的迟发性毒性尚未被证明。 作者报告1例患镰状细胞性贫血的非洲女孩,在成功的同种BMT后,发生原发性卵巢衰竭的病例。规定方案中,包括14天内给予马利兰总量为16mg/kg和环磷酰胺总量200mg/kg。移植前一月,病人年龄为13岁2个月,为临床性青春前期。检测提示为垂体-性腺轴青春前成熟。雌二醇为44pmol/L(正常值:37～92),铁蛋白:329μg/L(正常值:10～300)。到14岁9个月,仍无青春期临床征象,骨龄为12岁,LH和FSH基础血清水平分别为75mIU/L和94mIU/L,提示有严重性的原发
Bone marrow transplantation (BMT) allows the sick sickle cell disease to be cured in young patients. However, the delayed toxicity of marilan and cyclophosphamide administered as part of the prescribed regimen has not yet been demonstrated. The authors report an African girl with sickle cell anemia who developed primary ovarian failure following successful BMT. The prescribed regimen includes a total of 16 mg / kg of marilan and 200 mg / kg of cyclophosphamide administered within 14 days. One month prior to transplantation, the patient was 13 years old and 2 months old and was a clinical pre-teen. Test tips for the pituitary - gonadal axis premature ejaculation. Estradiol was 44 pmol / L (normal: 37-92) and ferritin: 329 μg / L (normal: 10-300). By the age of 14 years and 9 months, there were still no clinical signs of adolescence with a bone age of 12 years and basal serum levels of LH and FSH of 75 mIU / L and 94 mIU / L, respectively, suggesting a serious primary