Acquired hemophilia A in a patient with systemic lupus erythematosus.
Acquired hemophilia A in a patient with systemic lupus erythematosus.
复制标题
系统性红斑狼疮患者获得性血友病 A。
DOI：
10.2169/internalmedicine.40.541
复制
发表时间：
2001
影响因子：
1.2
通讯作者：
T. Naruse
中科院分区：
文献类型：
--
作者：
Tomomi Ishikawa;N. Tsukamoto;M. Suto;H. Uchiumi;H. Mitsuhashi;A. Yokohama;Akira Maesawa;Y. Nojima;T. Naruse
1例系统性红斑狼疮(SLE)患者发展为获得性血友病A。患者，24岁，日本女性，因拔牙后失控出血而转诊至本院。实验室检查显示APTT延长(116秒)，凝血因子VIII活性降低(2.8%)，凝血因子VIII抑制物滴度为46.5贝塞斯达单位/毫升。输注凝血酶原复合物浓缩物和活化的凝血酶原复合体浓缩物，然后应用强的松龙和环磷酰胺，成功地止血，降低了凝血因子VIII抑制物水平。获得性血友病A是一种罕见但致命的疾病。快速诊断和引入适当的治疗方法至关重要。
A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.