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儿童继发性免疫性血小板减少症:来自两个西班牙中心的大型队列的特征和结局。

Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres.

机构信息

Pediatric Hematology Department, Hospital Sant Joan de Déu Barcelona, Esplugues de Llobregat, Barcelona, Spain.

Institut de Recerca, Pediàtrica Hospital Sant Joan de Déu de Barcelona (IRP-HSJD0), Barcelona, Spain.

出版信息

Acta Paediatr. 2021 Jun;110(6):1952-1958. doi: 10.1111/apa.15765. Epub 2021 Mar 16.

DOI:10.1111/apa.15765
PMID:33460494
Abstract

AIM

To evaluate the incidence and outcome of secondary immune thrombocytopenia (ITP) in a large cohort of paediatric Spanish patients.

METHODS

A retrospective observational study was conducted in two paediatric University hospitals in Spain between 2009 and 2019, which included children from 4 months to 18 years old diagnosed with ITP. Data were recorded from clinical charts: gender, age at diagnosis, coexisting condition and associated characteristics, outcome and treatment.

RESULTS

Secondary ITP was diagnosed in 87 out of 442 patients (19.6%). Post-immunisation ITP was seen in younger children. The onset of secondary ITP to autoimmune diseases (AD) and immunodeficiencies (ID) was at an older age and had more tendency to be insidious, and platelet level was higher than primary ITP. Mean time from ITP onset to AD diseases or ID diagnosis was 1.2 and 2.6 years, respectively. Whereas the cumulative incidence of remission was significantly higher in post-immunisation and post-viral infection (compared with primary ITP patients), it was worse in AD and ID patients.

CONCLUSIONS

Identification of secondary ITP is important as it predicts outcome. Most of them are diagnosed at ITP onset, but AD diseases and ID should be ruled out periodically as they are usually identified later.

摘要

目的

评估西班牙儿科大样本队列中继发性免疫性血小板减少症(ITP)的发病率和结局。

方法

本研究为西班牙两家儿科大学医院在 2009 年至 2019 年期间开展的回顾性观察性研究,纳入 4 月龄至 18 岁诊断为 ITP 的儿童患者。从临床病历中记录数据:性别、诊断时年龄、共存疾病和相关特征、结局和治疗情况。

结果

442 例患者中诊断为继发性 ITP 87 例(19.6%)。儿童在疫苗接种后更易发生 ITP。继发性 ITP 继发于自身免疫性疾病(AD)和免疫缺陷(ID)的发病年龄更大,且更倾向于隐匿性发病,血小板计数高于原发性 ITP。从 ITP 发病到 AD 疾病或 ID 诊断的平均时间分别为 1.2 年和 2.6 年。虽然与原发性 ITP 患者相比,疫苗接种后和病毒感染后患者的缓解累积发生率显著更高,但 AD 和 ID 患者的缓解率更差。

结论

识别继发性 ITP 很重要,因为它可预测结局。大多数患者在 ITP 发病时被诊断,但应定期排除 AD 疾病和 ID,因为这些疾病通常在后期被发现。

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Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres.儿童继发性免疫性血小板减少症:来自两个西班牙中心的大型队列的特征和结局。
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