Vazzana Natale, Scarti Luca, Beltrame Chiara, Picchi Antonella, Taccetti Gianni, Fortini Alberto
Department of Internal Medicine, "S. Giovanni di Dio" Hospital, Via di Torregalli, 3-50143 Florence, Italy.
Department of Clinical Chemistry, "S. Giovanni di Dio" Hospital, Via di Torregalli, 3-50143 Florence, Italy.
Case Rep Hematol. 2014;2014:626831. doi: 10.1155/2014/626831. Epub 2014 Nov 6.
Development of autoantibodies against coagulation factors is an uncommon bleeding disorder associated with cancer, autoimmune conditions, pregnancy, or no apparent disease. Spontaneous FVIII inhibitors are the most frequently encountered; those against FXI have been only anecdotally reported. We report a case of acquired FXI inhibitor presenting as fatal intracranial spontaneous bleeding in an elderly patient with history of cancer and previous transfusions. Few cases of acquired FXI inhibitor have been reported in association with connective tissue disease, cancer, or surgery. Bleeding includes mucocutaneous bleeding, postsurgical hemorrhage, or life-threatening events. Treatment consists of arresting the bleeding and inhibitor eradication. High degree of suspicion is essential to promptly diagnose and treat this uncommon condition.
针对凝血因子的自身抗体的产生是一种罕见的出血性疾病,与癌症、自身免疫性疾病、妊娠或无明显疾病相关。自发性FVIII抑制剂最为常见;针对FXI的抑制剂仅有零星报道。我们报告一例获得性FXI抑制剂病例,该病例表现为一名有癌症病史和既往输血史的老年患者发生致命性颅内自发性出血。与结缔组织病、癌症或手术相关的获得性FXI抑制剂病例报道较少。出血包括皮肤黏膜出血、术后出血或危及生命的事件。治疗包括止血和消除抑制剂。高度怀疑对于及时诊断和治疗这种罕见疾病至关重要。