Patel Neeral, Wyrko Zoe, Naqvi Syeda, Croft Adam P
Department of Geriatric Medicine, Queen Elizabeth Hospital, Birmimgham, UK.
Sandwell General Hospital, West Bromwich, UK.
BMJ Case Rep. 2014 Nov 20;2014:bcr2014206911. doi: 10.1136/bcr-2014-206911.
We describe the case of a 79-year-old man who presented 'off legs' with acute right leg pain. He was initially treated for suspected cellulitis but subsequently found to have spontaneous soft tissue bleeding into the right thigh. He was eventually diagnosed with idiopathic acquired haemophilia A and treated with activated prothrombin complex concentrates to control acute bleeding followed by immunosuppressant therapy. Acquired haemophilia A is a potentially life-threatening and under-recognised bleeding disorder that results from the immune-mediated development of autoantibodies directed against coagulation factor VIII. The disease is more common in elderly individuals where early recognition and treatment is compounded by the presence of other comorbid conditions, including other potential causes of bleeding. These confounding factors and lack of awareness among non-specialists accounts for the delay in diagnosis that is common in this disease and contributes to the persistently high mortality in this age group.
我们描述了一名79岁男性的病例,他出现“下肢不适”并伴有急性右腿疼痛。他最初因疑似蜂窝织炎接受治疗,但随后发现右大腿出现自发性软组织出血。他最终被诊断为获得性血友病A,并接受了活化凝血酶原复合物浓缩剂治疗以控制急性出血,随后进行了免疫抑制治疗。获得性血友病A是一种潜在危及生命且未得到充分认识的出血性疾病,由针对凝血因子VIII的自身抗体的免疫介导产生所致。该疾病在老年人中更为常见,由于存在其他合并症,包括其他潜在出血原因,使得早期识别和治疗变得更加复杂。这些混杂因素以及非专科医生缺乏认识导致了该病常见的诊断延迟,并导致该年龄组死亡率持续居高不下。