Department of Haematology, Royal Infirmary of Edinburgh, Edinburgh, Scotland
Department of Haematology, Royal Infirmary of Edinburgh, Edinburgh, Scotland.
BMJ Case Rep. 2022 Aug 29;15(8):e244928. doi: 10.1136/bcr-2021-244928.
We detail the case of a man in his 80s who was a Jehovah's Witness, presenting to hospital for the second time in 1 week with atraumatic, severe bruising affecting his right thigh and flank. He was subsequently diagnosed with idiopathic acquired haemophilia A (AHA) and was urgently treated with recombinant factor concentrate and immunosuppressive therapy. Management of his bleeding disorder and resultant severe anaemia was adapted in line with his religious beliefs. AHA is a rare bleeding disorder which should be considered in patients with an isolated prolonged activated partial thromboplastin time and a history of recent or acute bleeding. Prompt diagnosis and management are essential as delays may result in increased mortality. Given that this patient declined blood transfusion, management of his bleeding disorder presented a unique challenge.
我们详细介绍了一位 80 多岁的男性患者，他是一名耶和华见证人，在一周内因无创伤性、严重瘀伤第二次到医院就诊，瘀伤影响他的右大腿和侧腹。随后他被诊断为特发性获得性血友病 A（AHA），并紧急接受了重组因子浓缩物和免疫抑制治疗。根据他的宗教信仰，对他的出血性疾病和由此导致的严重贫血进行了管理。AHA 是一种罕见的出血性疾病，应考虑在具有孤立性延长的活化部分凝血活酶时间和近期或急性出血史的患者中。及时诊断和管理至关重要，因为延迟可能导致死亡率增加。鉴于该患者拒绝输血，他的出血性疾病的管理带来了独特的挑战。
