Acquired Hemophilia A: Current Guidance and Experience from Clinical Practice.
Acquired Hemophilia A: Current Guidance and Experience from Clinical Practice.
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DOI：
10.2147/jbm.s284804
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发表时间：
2022
影响因子：
2
通讯作者：
中科院分区：
文献类型：
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作者：
在获得性 A 型血友病 (AHA) 中，凝血因子 VIII (FVIII) 的自身抗体中和 FVIII 活性，导致潜在的严重出血素质，从而导致高发病率和死亡率。这种疾病很少见，主要发生在60岁以上的成年人或产后期。对于没有个人或家族出血史的新发出血患者应怀疑该诊断，并且可以通过 FVIII 抑制剂的特异性检测来确认。治疗包括减少出血的止血疗法和重建对 FVIII 免疫耐受的免疫调节策略。关于难治性疾病治疗的数据有限，主要基于小病例系列。登记研究为最佳止血疗法和初始免疫抑制疗法提供了共识指南。需要进行更多研究来评估新型止血剂并开发生物标志物来对治疗进行风险分层，同时限制不良事件。
In acquired hemophilia A (AHA), autoantibodies to coagulation factor VIII (FVIII) neutralize FVIII activity leading to a potentially severe bleeding diathesis that carries a high rate of morbidity and mortality. This disorder is rare and occurs mainly in adults over 60 years of age or in the postpartum period. The diagnosis should be suspected in patients with new-onset bleeding without a personal or family history of bleeding and can be confirmed via specific assays for FVIII inhibitors. Treatment involves both hemostatic therapies to decrease bleeding and immune modulation strategies to re-establish immune tolerance to FVIII. There are limited data on treatment for refractory disease, based mostly on small case series. Registry studies have informed consensus guidelines for optimal hemostatic therapies and initial immunosuppressive therapies. Additional studies are needed to evaluate novel hemostatic agents and develop biomarkers to risk-stratify treatment while limiting adverse events.