Department of Hematology and Medical Oncology, Kyung Hee University, College of Medicine, Seoul, Republic of Korea.
Medicine (Baltimore). 2024 Aug 2;103(31):e39166. doi: 10.1097/MD.0000000000039166.
Acquired hemophilia A (AHA) is a rare autoimmune disease caused by an antibody that inhibits coagulation factor VIII activity. More than half of patients with AHA cannot identify underlying disorders. The remaining patients are associated with malignancies, autoimmune diseases, skin diseases, infections, and medications. Here, we present a case of 56-year-old Korean man with underlying hypertension, dyslipidemia, and diabetes mellitus who developed AHA following the second dose of BNT162b2 COVID-19 vaccination.
He presented with a large 20 × 30 cm-sized hematoma along the psoas muscle and intracranial hemorrhage, necessitating intensive care with mechanical ventilation and continuous renal replacement therapy. Laboratory testing demonstrated that activated partial thromboplastin time and prothrombin times were 74.7 seconds (normal range 29-43 seconds) and 17.2 seconds (normal range 12.5-14.7 seconds), respectively.
Laboratory tests confirmed AHA with undetectable factor VIII activity (<1.5%) and a positive factor VIII antibody with a titer of 8.49 Bethesda units/mL.
Recombinant factor VIIa (NovoSeven®) was administered every 2 hours to control the bleeding, alongside immunosuppression with methylprednisolone 1 mg/kg daily and cyclophosphamide 2 mg/kg daily to eliminate the autoantibody.
Despite the treatments, the patient developed sepsis and succumbed 14 weeks after admission.
This rare case underscores the importance of monitoring for AHA following COVID-19 vaccination. Although the benefits outweigh the risks of vaccination, AHA should be considered in the differential diagnosis of unusual bleeding following the vaccinations. Early diagnosis and management before severe bleeding are critical for successfully controlling life-threatening bleeding.
获得性血友病 A（AHA）是一种罕见的自身免疫性疾病，由抑制凝血因子 VIII 活性的抗体引起。超过一半的 AHA 患者无法确定潜在疾病。其余患者与恶性肿瘤、自身免疫性疾病、皮肤病、感染和药物有关。在这里，我们报告了一例 56 岁韩国男性，患有高血压、血脂异常和糖尿病，在接种第二剂 BNT162b2 COVID-19 疫苗后发生 AHA。
他出现了沿着腰肌的 20×30 厘米大小的血肿和颅内出血，需要进行重症监护，包括机械通气和持续肾脏替代治疗。实验室检测显示，活化部分凝血活酶时间和凝血酶原时间分别为 74.7 秒（正常范围 29-43 秒）和 17.2 秒（正常范围 12.5-14.7 秒）。
实验室检查证实 AHA 因子 VIII 活性不可检测（<1.5%），因子 VIII 抗体呈阳性，效价为 8.49 贝塞斯达单位/mL。
每 2 小时给予重组因子 VIIa（NovoSeven®）以控制出血，同时给予免疫抑制治疗，每日给予 1mg/kg 甲基强的松龙和 2mg/kg 环磷酰胺以消除自身抗体。
尽管进行了治疗，患者还是出现了败血症，并在入院后 14 周死亡。
这个罕见的病例强调了在 COVID-19 疫苗接种后监测 AHA 的重要性。虽然疫苗接种的益处大于风险，但在接种疫苗后出现异常出血时应考虑 AHA 的鉴别诊断。在发生危及生命的严重出血之前进行早期诊断和治疗对于成功控制危及生命的出血至关重要。
