Acquired Hemophilia A
Acquired Hemophilia A
复制标题
DOI：
10.1177/1076029613488937
复制
发表时间：
2015-01
期刊：
影响因子：
--
通讯作者：
Yan-hui Yang;F. Xue;H. Shi;Hongmei Wang;Lei Zhang;L. Ji;R. Yang
中科院分区：
文献类型：
--
作者：
Yan-hui Yang;F. Xue;H. Shi;Hongmei Wang;Lei Zhang;L. Ji;R. Yang
Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the autoantibody directed against factor VIII in patients without previous history of a bleeding disorder. We retrospectively analyzed the characteristics and outcomes of 49 patients with AHA diagnosed in our center from February 1994 to October 2012. Twenty-four patients with acute bleeding episodes were treated with prothrombin complex concentrate (PCC) at a relative low dose of 30 to -50 U/kg/d and achieved good outcomes without any adverse reaction. Corticosteroids alone or in combination with cyclophosphamide were used as the first-line therapy to eradicate the inhibitors. In 39 evaluable patients, 35 (89.7%) achieved complete remission (CR). This study demonstrates that when bypassing agents such as recombinant activated factor VII and activated PCCs are not affordable or available, low dose PCC is effective and safe to control acute bleeding in patients with AHA. First-line therapy achieved good outcomes with a CR rate of 89.7%.
