Shah Shubhangi, Tseng Michael, Durojaiye Ashimiyu
Internal Medicine, Virginia Commonwealth University School of Medicine, Richmond, USA.
Hematology and Medical Oncology, Virginia Commonwealth University School of Medicine, Richmond, USA.
Cureus. 2023 Aug 26;15(8):e44169. doi: 10.7759/cureus.44169. eCollection 2023 Aug.
Acquired hemophilia A (AHA) or factor VIII (FVIII) deficiency is caused by autoantibodies targeting FVIII in the blood coagulation pathway; it is a rare condition making it challenging to diagnose. A timely diagnosis is crucial, without which there is a risk of catastrophic bleeding. We report a case of a patient with a history of duodenal arteriovenous malformations, previously on apixaban, who presented with four days of melena. On admission he was found to have a hemoglobin of 5.7 and elevated partial thromboplastin time (PTT), promoting further workup showing FVIII levels of <1%, with a mixing study that failed to correct suggesting the presence of inhibitors against FVIII. Other characteristics of this patient's cases included controlled rheumatoid arthritis without detectable rheumatoid factor or increased erythrocyte sedimentation rate (ESR). The patient was initially treated with prednisone and intravenous immunoglobulins, but an insufficient response prompted the initiation of recombinant factor VII, rituximab, and cyclophosphamide during hospitalization.
获得性血友病A(AHA)或因子VIII(FVIII)缺乏症是由针对凝血途径中FVIII的自身抗体引起的;这是一种罕见疾病,诊断具有挑战性。及时诊断至关重要,否则有发生灾难性出血的风险。我们报告一例有十二指肠动静脉畸形病史、之前服用阿哌沙班的患者,该患者出现了四天的黑便。入院时发现他的血红蛋白为5.7,部分凝血活酶时间(PTT)升高,进一步检查显示FVIII水平<1%,混合试验未能纠正,提示存在针对FVIII的抑制剂。该患者病例的其他特征包括类风湿关节炎得到控制,类风湿因子未检测到,红细胞沉降率(ESR)未升高。患者最初接受泼尼松和静脉注射免疫球蛋白治疗,但反应不足促使在住院期间开始使用重组因子VII、利妥昔单抗和环磷酰胺。