误诊为特发性血小板减少性紫癜的儿童再生障碍性贫血临床分析

Clinical analysis of children with aplastic anemia misdiagnosed as ITP

目的通过对误诊为特发性血小板减少性紫癜(ITP)的再生障碍性贫血(再障)患儿临床特点和误诊原因的分析,提高临床医师对儿童再障的认识,减少早期误诊。方法收集我院1992年1月-2010年8月收治的曾误诊为ITP、入院后确诊为再障的20例患儿的临床资料,分别按照实验室检查、疗效及误诊原因进行回顾性分析。结果在所有再障患儿中,误诊为ITP者占16%,在被诊断为ITP时:(1)多数患儿已经出现明显的外周血二系或三系下降,而并非单纯血小板减少。(2)5例患儿行骨髓检查,但结果并不符合ITP骨髓象。(3)绝大部分患儿未进行必要的血小板抗体检查,或检查结果并不典型。(4)按ITP进行糖皮质激素和大剂量免疫球蛋白治疗,疗效欠佳,未能进一步检查以核实诊断。(5)所有患儿均不符合ITP诊断标准,经过进一步检查均符合再障诊断标准。经抗胸腺细胞免疫球蛋白、环胞素A和雄性激素等治疗后,参照再障疗效标准,总有效率和显效率分别为75%和62%。结论再障和ITP均存在明显的血小板减少和出血倾向,ITP发病率明显高于再障,临床容易发生误诊。因此,临床上发现血小板降低,在考虑ITP诊断时,必须同时考虑可导致血小板减少的其他疾病的可能性,严格参照中华医学会制定的"再生障碍性贫血诊断标准"和"特发性血小板减少性紫癜诊断标准",以明确诊断和进行必要的鉴别诊断。

Objective To better recognize aplastic anemia （AA） and avoid misdiagnosis of it in early stage by clinical manifestations. Methods Twenty children with AA from January of 1992 to August of 2010 had been misdiagnosed as ITP. They were retrospectively analyzed based on their clinical features, laboratory findings, response to treatment and misdiagnosis reasons. Results According to clinical data, misdiagnosis rate was 16.0%. Most patients had been found bicytopenia or pancytopenia rather than simple thrombocytopenia when they were diagnosed as ITP. The results of bone marrow in 5 patients did not support the diagnosis of ITP. Most patients did not detect platelet antibody, or the results were not typical. Further examinations were not checked to confirm the diagnosis when the response to glucocorticoid and high-dose immunoglobulin treatment was poor. All patients were confirmed as AA rather than ITP by further examinations. The total efficacy rate and significant efficacy rate were 75.0% and 62.5% respectively by the treatment of antithymocyte （ATG）, cyclosporin A（CSA）and Androgen. Conclusion AA and ITP are all characterized by thrombocytopenia and bleeding tendency, which were more significant in ITP. Therefore, other diseases with thrombocytopenia should be considered. Meanwhile, the diagnostic criteria of AA and ITP established by Chinese Medical Association should also be taken strictly to make a definite diagnosis.