Pathirana Upul G P, Gunawardena Nirmali, Abeysinghe Hiranya, Copley Hannah Charlotte, Somarathne M G Dhammika
General Medical Unit, Teaching Hospital, Kandy, Sri Lanka.
J Med Case Rep. 2014 Dec 29;8:469. doi: 10.1186/1752-1947-8-469.
Acquired haemophilia A is a rare life- and limb-threatening bleeding disorder if left untreated. Autoimmune thyroiditis is an autoimmune disorder that can be rarely associated with acquired haemophilia. Here we report a case of a 60-year-old woman presenting with cutaneous and muscle haematomas secondary to acquired haemophilia A in association with autoimmune thyroiditis, who was successfully treated with recombinant activated factor VII and immunosuppression.
A 60-year-old Sri Lankan woman with a background of longstanding hypothyroidism, diabetes mellitus, hypertension, hyperlipidaemia and bronchial asthma developed spontaneous cutaneous purpura and a limb-threatening intramuscular haematoma. Initial coagulation screening revealed prolonged activated partial thromboplastin time of 66.4 seconds (normal range 26 to -36 seconds) and time-dependent inhibitors against factor VIII. She had positive antinuclear antibody and antithyroid peroxidase (microsomal) antibody titre of over 1/80 and 1000IU/mL respectively. The diagnosis was therefore made of acquired haemophilia A in association with autoimmune thyroiditis. Acute limb-threatening bleeding was managed with recombinant activated factor VII (NovoSeven®). Immunosuppressive treatment consisting of oral prednisone 60mg/day and cyclophosphamide 100mg/day was administered in order to remove the factor VIII inhibitor. This treatment led to normalisation of her haemostatic parameters. This case illustrates a very rare association of acquired haemophilia and autoimmune thyroiditis as well as the importance of considering acquired haemophilia as a differential diagnosis of spontaneous bleeding.
Acquired haemophilia should be considered in the differential diagnosis of unexplained bleeding in adults. Treatment of the acute coagulopathy with recombinant activated factor VII and immunosuppressive therapy was successful in this case.
获得性血友病A是一种罕见的、若不治疗会危及生命和肢体的出血性疾病。自身免疫性甲状腺炎是一种自身免疫性疾病，很少与获得性血友病相关。在此，我们报告一例60岁女性，因获得性血友病A继发皮肤和肌肉血肿，并伴有自身免疫性甲状腺炎，经重组活化凝血因子VII和免疫抑制治疗成功治愈。
一名60岁的斯里兰卡女性，有长期甲状腺功能减退、糖尿病、高血压、高脂血症和支气管哮喘病史，出现自发性皮肤紫癜和危及肢体的肌内血肿。初始凝血筛查显示活化部分凝血活酶时间延长至66.4秒（正常范围26至36秒），且存在针对凝血因子VIII的时间依赖性抑制剂。她的抗核抗体和抗甲状腺过氧化物酶（微粒体）抗体滴度分别呈阳性，超过1/80和1000IU/mL。因此诊断为获得性血友病A合并自身免疫性甲状腺炎。急性危及肢体的出血采用重组活化凝血因子VII（诺其®）治疗。给予口服泼尼松60mg/天和环磷酰胺100mg/天的免疫抑制治疗，以消除凝血因子VIII抑制剂。该治疗使她的止血参数恢复正常。本病例说明了获得性血友病与自身免疫性甲状腺炎的罕见关联，以及将获得性血友病作为自发性出血鉴别诊断的重要性。
成人不明原因出血的鉴别诊断中应考虑获得性血友病。本病例中，用重组活化凝血因子VII治疗急性凝血病并结合免疫抑制疗法取得了成功。
