Guinan E C, Tarbell N J, Tantravahi R, Weinstein H J
Department of Pediatric, Dana-Farber Cancer Institute, Boston, MA 02115.
Blood. 1989 Feb;73(2):619-22.
Therapeutic options for children with de novo or secondary myelodysplastic syndromes (MDSs) are limited. We report the outcome of eight pediatric patients (median age 12 years, range 3 to 19 years) with myelodysplasia who underwent allogeneic bone marrow transplantation between 1984 and 1987. Two of the eight children had developed secondary myelodysplasia after alkylating agent-based combination chemotherapy. Five patients had clonal chromosomal abnormalities, including four patients with monosomy 7. Seven of eight patients engrafted. Two of these seven subsequently died of complications of acute or chronic graft-v-host disease (GVHD), and a third patient died at 21 months of pulmonary fibrosis. None of the patients have had recurrence of disease. The four surviving patients remain in complete remission at a median follow-up of 19 months (range 10 to 44 months).
初发或继发性骨髓增生异常综合征（MDS）患儿的治疗选择有限。我们报告了1984年至1987年间接受异基因骨髓移植的8例骨髓发育异常儿科患者（中位年龄12岁，范围3至19岁）的治疗结果。8名儿童中有2名在基于烷化剂的联合化疗后发生继发性骨髓发育异常。5例患者存在克隆性染色体异常，其中4例为7号染色体单体。8例患者中有7例植入成功。这7例中的2例随后死于急性或慢性移植物抗宿主病（GVHD）并发症，第3例患者在21个月时死于肺纤维化。所有患者均无疾病复发。4名存活患者在中位随访19个月（范围10至44个月）时仍处于完全缓解状态。
