Internal Medicine, Hospital Beatriz Angelo, Loures, Lisboa, Portugal
Internal Medicine, Hospital Beatriz Angelo, Loures, Lisboa, Portugal.
BMJ Case Rep. 2022 Apr 8;15(4):e248701. doi: 10.1136/bcr-2021-248701.
Acquired haemophilia A is a rare condition defined by the presence of coagulation inhibitors, which are autoantibodies directed against factor VIII that interfere with its activity. We report a case of a 69-year-old woman that presented with knee haemarthrosis followed by spontaneous retroperitoneal haematoma. On coagulation studies, she presented normal prothrombin time with prolonged activated partial thromboplastin time non-correcting on mixture test, low titers of factor VIII and was detected factor VIII inhibitor that led to diagnosis of acquired haemophilia A. She was managed with supportive measures to control haemorrhage and immunosuppressive therapy to eradicate inhibitors, initially with corticosteroids, with partial transitory response, after which she developed new spontaneous haematomas. Rituximab was started at that time, with a good outcome. The additional aetiological study identified autoimmune thyroiditis and autoimmune pangastritis, an association rarely described in literature.
获得性血友病 A 是一种罕见的疾病，其特征是存在凝血抑制剂，即针对因子 VIII 的自身抗体，从而干扰其活性。我们报告了一例 69 岁女性病例，该患者最初表现为膝关节血肿，随后自发性腹膜后血肿。在凝血研究中，她的凝血酶原时间正常，但活化部分凝血活酶时间延长，混合试验不能纠正，VIII 因子滴度低，且检测到 VIII 因子抑制剂，从而确诊为获得性血友病 A。她接受了支持性治疗以控制出血和免疫抑制治疗以消除抑制剂，最初使用皮质类固醇，但反应短暂，之后她又出现新的自发性血肿。此时开始使用利妥昔单抗治疗，效果良好。进一步的病因研究发现自身免疫性甲状腺炎和自身免疫性全胃炎，这种关联在文献中很少有描述。
