Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry
Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry
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DOI：
10.1111/j.1471-0528.2012.03469.x
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发表时间：
2012-11-01
影响因子：
5.8
通讯作者：
Collins, P.
中科院分区：
文献类型：
--
作者：
Tengborn, L.;Baudo, F.;Collins, P.
请将本文引用为：Tengborn L，Baudo F，Huth-Kuhne A，Knoebl P，Levesque H，Marco P，Pellegrini F，Nemes L，柯林斯P代表EACH 2登记研究贡献者。妊娠相关获得性血友病A：来自欧洲获得性血友病（EACH 2）登记的结果。BJOG 2012;119：15291537。目的欧洲获得性血友病登记中心（EACH 2）收集了获得性血友病A（AHA）女性患者的人口统计学资料、诊断、基础疾病、出血特征、治疗和结局。AHA是一种罕见且通常严重的出血性疾病，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、泛欧登记研究。在13个欧洲国家共有117个血友病中心。人群妊娠相关AHA。方法使用基于网络的电子病例报告表报告数据。诊断基于活化部分凝血活酶时间延长、凝血因子VIII水平降低和抑制物测定阳性。主要观察指标表现特征、诊断时间、止血治疗和结果、免疫抑制治疗和结果。结果EACH 2登记研究（n = 501）记录了42例（8.4%）与围产期相关的AHA病例，诊断时的中位因子VIII水平为2.5（范围025）IU/dl，抑制剂滴度为7.8（范围0.7348）BU/ml。八名女性的产前抑制剂明显。分娩后至AHA诊断的时间为89（范围21120）天。20/23例（87%）接受治疗的女性的一线止血治疗成功。17/18例（94%）接受旁路药物治疗的女性出血事件消退，29/39例（74%）接受一线免疫抑制治疗的女性完全缓解。两名婴儿出现产后出血，表明抗体经胎盘转移。所有女性在末次随访时均存活。结论虽然罕见，但妊娠相关AHA可能导致严重的出血相关的发病率。一旦确诊，妇女对旁路药物和免疫抑制剂的止血治疗反应良好。对围产期AHA的认识需要提高，以促进快速和适当的管理。
Please cite this paper as: Tengborn L, Baudo F, Huth-Kuhne A, Knoebl P, Levesque H, Marco P, Pellegrini F, Nemes L, Collins P on behalf of the EACH2 registry contributors. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG 2012;119:15291537. 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 025) IU/dl and inhibitor titre of 7.8 (range 0.7348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21120) 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.