Clement-De Boers A, Oostdijk W, Van Weel-Sipman M H, Van den Broeck J, Wit J M, Vossen J M
Department of Pediatrics, University Hospital, Leiden, The Netherlands.
J Pediatr. 1996 Oct;129(4):544-50. doi: 10.1016/s0022-3476(96)70119-1.
To analyze final height and hormonal function in long-term survivors of bone marrow transplantation (BMT).
Group 1 consisted of 16 patients (10 boys) with a hematologic malignancy, mostly leukemia, conditioned for BMT with total body irradiation (TBI), 7.5 to 12 Gy, and cyclophosphamide. Group 2 consisted of 14 patients (9 boys) with severe aplastic anemia, conditioned with chemotherapy only.
In group 1, patients achieved a reduced final height after BMT. The difference between the height standard deviation score (SDS) at BMT and the height SDS at final height was -1.96 (0.82) SDS in boys and -0.92 (0.71) SDS in girls (p = 0.0001, and p = 0.02 respectively). Final height was also lower than target height (boys, p = 0.01; girls, p = 0.03). Prepubertal growth in the first 3 years after BMT was normal but pubertal height gain was decreased. The patients in group 2 achieved normal height. Thyroid function and adrenal function were normal in all patients, and no growth hormone deficiency was detected. Serum follicle-stimulating hormone values after BMT were increased in all group 1 patients, with return to normal in two patients. Serum luteinizing hormone values were increased in all group 1 girls, with recovery in one girl. Normal serum luteinizing hormone values and spontaneous puberty were found in all group 1 boys. In group 2, disturbances in gonadotropins were seen only in three boys and two girls.
In patients treated in childhood with BMT after chemotherapy and TBI with 7.5 Gy or more, final height is compromised because of blunted growth in puberty. Patients who had not received TBI suffered no height loss. In the majority of patients, the combination of chemotherapy and TBI also resulted in irreversible disturbances of gonadal function.
分析骨髓移植(BMT)长期存活者的最终身高和激素功能。
第1组由16例血液系统恶性肿瘤患者(10例男孩)组成,主要为白血病,接受全身照射(TBI)7.5至12 Gy及环磷酰胺预处理进行BMT。第2组由14例严重再生障碍性贫血患者(9例男孩)组成,仅接受化疗预处理。
在第1组中,患者BMT后最终身高降低。BMT时身高标准差评分(SDS)与最终身高时身高SDS的差值,男孩为-1.96(0.82)SDS,女孩为-0.92(0.71)SDS(分别为p = 0.0001和p = 0.02)。最终身高也低于目标身高(男孩,p = 0.01;女孩,p = 0.03)。BMT后前3年的青春期前生长正常,但青春期身高增长减少。第2组患者身高正常。所有患者甲状腺功能和肾上腺功能正常,未检测到生长激素缺乏。第1组所有患者BMT后血清促卵泡激素值升高,2例患者恢复正常。第1组所有女孩血清黄体生成素值升高,1例女孩恢复正常。第1组所有男孩血清黄体生成素值正常且有自然青春期。在第2组中,仅3例男孩和2例女孩出现促性腺激素紊乱。
儿童期接受7.5 Gy或更高剂量化疗和TBI后进行BMT的患者,由于青春期生长迟缓,最终身高受到影响。未接受TBI的患者身高未降低。在大多数患者中,化疗和TBI的联合应用还导致性腺功能不可逆转的紊乱。