Outcome of CARE: a 6‐year national registry of acquired haemophilia A in China

Summary Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by autoantibodies directed against the functional epitopes of coagulation factor VIII (FVIII). Its management relies on prompt diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. China Acqui...

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Veröffentlicht in:British journal of haematology 2019-12, Vol.187 (5), p.653-665
Hauptverfasser: Sun, Boyang, Xue, Feng, Feng, Ying, Sun, Jing, Yu, Ziqiang, Hou, Ming, Zhang, Jingyu, Zeng, Xiaojing, Zhao, Yongqiang, Lian, Shimei, Huang, Meijuan, Niu, Ting, Cui, Zhongguang, Wu, Jingsheng, Yang, Renchi
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Sprache:eng
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Zusammenfassung:Summary Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by autoantibodies directed against the functional epitopes of coagulation factor VIII (FVIII). Its management relies on prompt diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. China Acquired Hemophilia Registry (CARE), a nationwide multicentre registry, was intended to survey the status of AHA and standardize its diagnosis and therapy in China. One hundred and eighty‐seven registered patients had an average age of 52 years. Diagnosis was delayed in 46·5% patients. There was a significant delay from diagnosis to immunosuppressive therapy in 68·3% patients. Bleeding control was significantly higher in patients treated with prothrombin complex concentrate (PCC) versus FVIII replacement therapy (84·6% vs. 34·4%; P 
ISSN:0007-1048
1365-2141
DOI:10.1111/bjh.16128