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持续性和慢性免疫性血小板减少症患儿的出血表现和处理:来自国际免疫性血小板减少症协作研究组(ICIS)的数据。

Bleeding manifestations and management of children with persistent and chronic immune thrombocytopenia: data from the Intercontinental Cooperative ITP Study Group (ICIS).

机构信息

Department of Pediatrics, Georgia Regents University, Augusta, GA 30912, USA.

出版信息

Blood. 2013 May 30;121(22):4457-62. doi: 10.1182/blood-2012-12-466375. Epub 2013 Apr 2.

DOI:10.1182/blood-2012-12-466375
PMID:23550040
Abstract

Long-term follow-up of children with immune thrombocytopenia (ITP) indicates that the majority undergo remission and severe thrombocytopenia is infrequent. Details regarding bleeding manifestations, however, remain poorly categorized. We report here long-term data from the Intercontinental Cooperative ITP Study Group Registry II focusing on natural history, bleeding manifestations, and management. Data on 1345 subjects were collected at diagnosis and at 28 days, 6, 12, and 24 months thereafter. Median platelet counts were 214 × 10(9)/L (interquartile range [IQR] 227, range 1-748), 211 × 10(9)/L (IQR 192, range 1-594), and 215 × 10(9)/L (IQR 198, range 1-598) at 6, 12, and 24 months, respectively, and a platelet count <20 × 10(9)/L was uncommon (7%, 7%, and 4%, respectively). Remission occurred in 37% of patients between 28 days and 6 months, 16% between 6 and 12 months, and 24% between 12 and 24 months. There were no reports of intracranial hemorrhage, and the most common site of bleeding was skin. In patients with severe thrombocytopenia we observed a trend toward more drug treatment with increasing number of bleeding sites. Our data support that ITP is a benign condition for most affected children and that major hemorrhage, even with prolonged severe thrombocytopenia, is rare.

摘要

儿童免疫性血小板减少症(ITP)的长期随访表明,大多数患者会缓解,严重血小板减少症很少见。然而,关于出血表现的详细信息仍分类不当。我们在此报告国际多中心 ITP 研究组注册研究 II 的长期数据,重点关注自然史、出血表现和治疗。在诊断时以及此后的 28 天、6 个月、12 个月和 24 个月收集了 1345 名受试者的数据。中位血小板计数分别为 214×10(9)/L(四分位距[IQR]227,范围 1-748)、211×10(9)/L(IQR 192,范围 1-594)和 215×10(9)/L(IQR 198,范围 1-598)在 6、12 和 24 个月时,血小板计数<20×10(9)/L 的情况并不常见(分别为 7%、7%和 4%)。在 28 天至 6 个月之间,37%的患者缓解,6 个月至 12 个月之间缓解 16%,12 个月至 24 个月之间缓解 24%。没有颅内出血的报告,最常见的出血部位是皮肤。在严重血小板减少症患者中,我们观察到随着出血部位数量的增加,药物治疗的趋势增加。我们的数据支持大多数受影响的儿童 ITP 是一种良性疾病,即使存在长期严重血小板减少症,也很少发生重大出血。

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