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目的探讨小儿骨髓增生异常综合征(MDS)的临床特点。方法回顾性分析同济医学院附属同济医院1991～2005年间收治的94例小儿MDS的幼稚前体细胞异常定位(ALIP)、血清乳酸脱氢酶(LDH)、血红蛋白F(HbF)、血清铁蛋白(SF)以及细胞遗传学与预后的关系。结果94例中难治性贫血(RA)48例(51.1%),难治性贫血伴原始细胞增多(RAEB)26例(27.7%),转化中的难治性贫血伴原始细胞转化增多(RAEBt)20例(21.3%)。44.0%的病例(11/25)检出ALIP,随访中5例转为白血病。9.6%病例(9/94)伴有嗜酸性粒细胞增多,随访中的7例全部死亡,平均存活时间10.5个月。高危组的SF和LDH明显高于低危组,LDH<300U/L组平均存活时间明显长于LDH≥300U/L组。42.9%(9/21)病例伴有细胞遗传学改变,55.6%(5/9)转为白血病。结论小儿MDS伴有SF和LDH的明显升高以及伴有嗜酸性粒细胞增多者预后不良,染色体核型分析对小儿MDS的诊断、预后评估有重要价值。
Objective To investigate the clinical features of pediatric myelodysplastic syndrome (MDS). Methods A retrospective analysis was performed on 94 cases of infantile MDS in Tongji Hospital from 1991 to 2005 with abnormal precursor localization (ALIP), serum LDH, HbF and serum ferritin SF) and the relationship between cytogenetics and prognosis. Results In 94 cases, refractory anemia (RA) was found in 48 cases (51.1%), refractory anemia with rheumatoid arthritis (RAEB) in 26 cases (27.7%), and refractory anemia with increased primary cell transformation ) In 20 cases (21.3%). ALIP was detected in 44.0% of the cases (11/25), and 5 of the follow-up were converted to leukemia. 9.6% of cases (9/94) were accompanied by eosinophilia. All 7 patients died at follow-up, with an average survival of 10.5 months. The SF and LDH in high risk group were significantly higher than those in low risk group, and the average survival time in LDH <300U / L group was significantly longer than that in LDH≥300U / L group. 42.9% (9/21) cases were associated with cytogenetic changes and 55.6% (5/9) were leukemias. Conclusions The significant increase of SF and LDH in children with MDS and the poor prognosis of patients with eosinophilia are associated with pediatric MDS. Chromosomal karyotype analysis is of great value in the diagnosis and prognosis evaluation of pediatric MDS.