Mayer E I, Dopfer R E, Klingebiel T, Scheel-Walter H, Ranke M B, Niethammer D
University Children's Hospital, Tübingen, Germany.
Pediatr Transplant. 1999 Feb;3(1):38-44. doi: 10.1034/j.1399-3046.1999.00006.x.
Total body irradiation and high-dose chemotherapy, applied as a preparatory regimen for bone marrow transplantation (BMT) in children with acute lymphoblastic leukemia (ALL), are particularly hazardous to the gonads and, in addition, can impair hypothalamo pituitary-gonadal control. Longitudinal data on pubertal development and gonadal function in these patients are limited. Twenty-one ALL patients (15 males, 6 females) who had successfully undergone allogeneic BMT before puberty (age at BMT: 3.4-12.3 yr) were followed up in University Children's Hospital, Tübingen, Germany over 2 (minimum) to 14 (maximum) years. Tanner development scores, serum testosterone and estradiol, basal follicle-stimulating hormone (FSH) and luteinizing hormone (LH) were analyzed. During pubertal age, the levels of FSH and LH rose consecutively, resulting in noticeably elevated serum concentrations in 100% and 89%, respectively, of boys older than 14 years and in 75% and 75%, respectively, of girls older than 13 years. Nevertheless, pubertal development has been normal in all patients except in one boy and two girls who required substitution with sexual steroids, as timely puberty (i.e. boys < 14 years, girls < 13 years) did not start. In males with normal puberty, testosterone levels, however, were found to be low-normal. In conclusion, after BMT preceded by total body irradiation for childhood ALL, gonadal function is impaired. Even if normal pubertal development occurs, deficiencies in long-term endocrine function cannot be ruled out. In view of the high FSH levels, the prognosis for fertility is doubtful.
全身照射和大剂量化疗作为急性淋巴细胞白血病（ALL）患儿骨髓移植（BMT）的预处理方案，对性腺危害极大，此外还会损害下丘脑 - 垂体 - 性腺轴的调控。关于这些患者青春期发育和性腺功能的纵向数据有限。21例在青春期前成功接受异基因BMT的ALL患者（15例男性，6例女性）（BMT时年龄：3.4 - 12.3岁）在德国图宾根大学儿童医院接受了2年（最短）至14年（最长）的随访。分析了坦纳发育评分、血清睾酮和雌二醇、基础促卵泡生成素（FSH）和促黄体生成素（LH）。在青春期，FSH和LH水平持续上升，14岁以上男孩中分别有100%和89%、13岁以上女孩中分别有75%和75%的血清浓度显著升高。然而，除了一名男孩和两名女孩需要用性类固醇替代治疗外，所有患者的青春期发育均正常，因为适时青春期（即男孩<14岁，女孩<13岁）未启动。在青春期发育正常的男性中，睾酮水平处于低正常范围。总之，儿童ALL患者在全身照射后进行BMT，性腺功能受损。即使出现正常的青春期发育，也不能排除长期内分泌功能缺陷。鉴于FSH水平较高，生育预后令人怀疑。
