Misdiagnosis of Chronic Thrombocytopenia in Childhood
Misdiagnosis of Chronic Thrombocytopenia in Childhood
复制标题
儿童慢性血小板减少症的误诊
DOI：
10.1097/00043426-200307000-00010
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发表时间：
2003
期刊：
影响因子：
--
通讯作者：
M. Dreyfus
中科院分区：
文献类型：
--
作者：
B. Bader;V. Proulle;Catherine Trichet;D. Debray;M. Gabolde;J. Yvart;M. Dreyfus
目的 最终诊断为非免疫性慢性血小板减少症的儿童通常被转诊至儿科血液诊所，并临时诊断为自身免疫性血小板减少性紫癜 (AITP)。作者的目的是在这些患者中确定血小板减少症机制的特征。患者和方法 作者对 1990 年至 2000 年间转诊至单一儿科血液中心的 7 名被误诊为慢性 AITP 的儿童（3 名男孩和 4 名女孩，年龄 5 个月至 7 岁）的病例记录进行了回顾性分析。结果 在这 7 名儿童中，转诊时的疑似诊断为 AITP，最终诊断为遗传性血小板减少症。所有患者均存在血小板和/或白细胞形态异常。提示遗传性血小板减少症的其他特征包括家族性血小板减少症病史 (2/7)、类固醇和/或静脉注射免疫球蛋白未能将血小板计数提高至正常水平 (5/7)，以及两名测试患者的 Indium-111 血小板周转中度增加。接受测试的四名儿童的血小板相关 IgG (PaIgG) 高于正常阈值；四名接受测试的儿童的直接单克隆抗体固定血小板抗原（MAIPA）试验呈阴性，两名男孩的血清试验呈阳性。骨髓检查显示巨核细胞数量正常（4/7）或升高（3/7）。结论 家族史和由经验丰富的人员进行的血细胞形态分析是区分孤立性慢性血小板减少症儿童 AITP 和遗传性血小板减少症的第一步。相比之下，骨髓检查和使用 MAIPA 测试寻找特定自身抗体的帮助不大。在考虑脾切除之前，应进行同位素血小板寿命研究（如有），以排除遗传性血小板减少症的诊断，特别是当类固醇和/或 IgG IV 给药未能提高血小板计数时。
Purpose Children ultimately diagnosed with nonimmune chronic thrombocytopenia are often referred to pediatric hematology clinics with a provisional diagnosis of autoimmune thrombocytopenic purpura (AITP). The authors' aim was to establish in these patients the features characterizing the mechanism of thrombocytopenia. Patients and Methods The authors performed a retrospective review of the case records of seven children (three boys and four girls, aged 5 months to 7 years) with misdiagnosed chronic AITP referred to a single pediatric hematology center between 1990 and 2000. Results In the seven children, the suspected diagnosis on referral was AITP and the final diagnosis was inherited thrombocytopenia. Abnormalities of platelets and/or leukocyte morphology were present in all of them. Other features suggestive of inherited thrombocytopenia included a history of familial thrombocytopenia (2/7), failure of steroids and/or intravenous immunoglobulins to raise the platelet count to normal levels (5/7), and moderate increase of Indium-111 platelet turnover in the two patients tested. Platelet-associated IgG (PaIgG) was above the normal threshold in the four children tested; the direct monoclonal antibody immobilization of platelet antigens (MAIPA) test was negative in the four children tested and the serum test was positive in two boys. Bone marrow examination revealed either a normal (4/7) or an elevated (3/7) number of megakaryocytes. Conclusions Family history and blood cell morphology analysis in experienced hands are the first steps in discriminating AITP from inherited thrombocytopenia in children with isolated chronic thrombocytopenia. In contrast, bone marrow examination and search for specific autoantibodies using the MAIPA test are of little help. An isotopic platelet life span study, when available, should be performed before considering splenectomy to exclude the diagnosis of inherited thrombocytopenia, especially when steroids and/or IgG IV administration failed to raise the platelet count.
影响因子：
5.1
作者：
SULLIVAN, KE;MULLEN, CA;WINKELSTEIN, JA
通讯作者：
WINKELSTEIN, JA