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妊娠相关性获得性血友病 A:来自欧洲获得性血友病(EACH2)登记处的结果。

Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry.

机构信息

Clinical Coagulation Research Unit, Skåne University Hospital, Malmö, Sweden.

出版信息

BJOG. 2012 Nov;119(12):1529-37. doi: 10.1111/j.1471-0528.2012.03469.x. Epub 2012 Aug 20.

Abstract

OBJECTIVE

The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII.

DESIGN

Prospective, multi-centre, large-scale, pan-European registry.

SETTING

A total of 117 haemophilia centres in 13 European countries.

POPULATION

Pregnancy-associated AHA.

METHODS

Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay.

MAIN OUTCOME MEASURES

Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome.

RESULTS

The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up.

CONCLUSIONS

Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.

摘要

目的

欧洲获得性血友病登记处(EACH2)收集了有关女性获得性血友病 A(AHA)的人口统计学、诊断、潜在疾病、出血特征、治疗和结局的数据,AHA 是一种罕见且常导致严重出血的疾病,由针对凝血因子 VIII 的自身抗体引起。

设计

前瞻性、多中心、大规模、泛欧登记处。

设置

来自 13 个欧洲国家的 117 个血友病中心。

人群

妊娠相关性 AHA。

方法

使用基于网络的电子病例报告表报告数据。诊断基于延长的活化部分凝血活酶时间、降低的凝血因子 VIII 水平和阳性抑制剂检测。

主要观察指标

表现特征、诊断时间、止血治疗和结局、免疫抑制治疗和结局。

结果

EACH2 登记处(n=501)记录了 42 例(8.4%)与围产期相关的 AHA,诊断时凝血因子 VIII 水平中位数为 2.5(范围 0-25)IU/dl,抑制剂滴度为 7.8(范围 0.7-348)BU/ml。8 例妇女产前存在抑制剂。产后 AHA 的诊断时间为 89(范围 21-120)天。23 例接受治疗的妇女中,一线止血治疗成功 20 例(87%)。17 例接受旁路制剂治疗的妇女出血发作得到缓解,18 例接受一线免疫抑制治疗的妇女完全缓解 29 例(74%)。2 例婴儿出现产后出血,提示抗体经胎盘转移。所有妇女在最后一次随访时均存活。

结论

尽管罕见,但妊娠相关性 AHA 可能导致严重的出血相关发病率。一旦诊断,女性对旁路制剂和免疫抑制的止血治疗反应良好。提高对围产期 AHA 的认识需要改善,以促进快速和适当的管理。

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