儿童再生障碍性贫血临床特点及临床分型探讨

目的探讨儿童再生障碍性贫血(再障)临床特点及临床分型。方法2003-01—2005-12在中山大学附属第二医院儿科确诊儿童再障50例,年龄2～15岁,其中37例接受免疫抑制治疗(IST),观察再障发病诱因、临床表现、血象、骨髓象变化特点、临床分型及治疗转归。结果86%的儿童再障为特发性,70%患儿病程≤6个月,按疾病严重程度分型,临床诊断重型再障(SAA)38例,轻型再障(MAA)12例,骨髓有核细胞增生程度除与病情严重程度相关外,还与取材部位、病程及是否接受IST等因素有关。特发性再障患儿强化IST(IIST)及单用环孢菌素A(CsA)治疗6个月有效率分别为78.3%(18/23)和40.0%(4/10,χ2=4.59,P<0.05),多数患儿于IIST后2～3个月起效,仅1例病程2年余的SAA于IIST后7个月达部分缓解。结论儿童再障多数为特发性,起病急,病情重,临床表现及血象改变严重程度与骨髓增生减低程度相关,特发性再障患儿IIST疗效显著优于单用CsA治疗者,宜按国际标准根据病情严重程度分为重型和轻型。

Clinical characteristics and classification of pediatric aplastic anemia

Objective To investigate clinical characteristics and classification of pediatric aplastic anemia(AA).Methods Fifty patients with acquired AA aged 2~15 years were analysed on etiology,clinical features,blood count,bone marrow aspiration and classification.Thirty-seven patients of them accepted immunosuppressive therapy(IST).Results Majority(86%)of the patients were idiopathic.The depressed marrow cellularity not only associated with severity of symptoms and blood counts but also with locations of the aspiration,courses of the disease,and IST.There were 38 severe and 12 moderate AA patients according to Camitta criteria.The rate of response to intensive IST(IIST)at 6 months was higher than that to CsA alone in the children with idiopathic AA(78.3% vs 40%,P<0.05).Most patients presented a response at 2 to 3 months after IIST,only one person got partial response at 7 months after IIST.Conclusion The majority of pediatric AA is idiopathic,acute and severe.The efficiency of IIST is better than that of CsA alone in patients with AA.AA should be classified as severe and moderate according to severity of disease.