Patra Soumya, Reddy Babu, Nagesh C M, Srinivas B C, Manjunath C N
Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India.
Heart Views. 2014 Jan;15(1):16-8. doi: 10.4103/1995-705X.132141.
Afibrinogenemia is a rare autosomal recessive bleeding disorder with an estimated prevalence of 1:1,000,000. Usual presentation of this disorder is spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures. Paradoxically, few patients with afibrinogenemia may also suffer from severe thromboembolic complications. The management of these patients is particularly challenging because they are not only at risk of thrombosis but also of bleeding. We are presenting a case of 33-year-old male patient of congenital afibrinogenemia who had two episodes myocardial infarction in a span of two years. The patient was managed conservatively with antiplatelet therapy and thrombolytic therapy was not given due to high risk for bleeding.
无纤维蛋白原血症是一种罕见的常染色体隐性出血性疾病,估计患病率为1:1,000,000。这种疾病的常见表现为自发性出血、轻微创伤后出血以及介入手术期间的过度出血。矛盾的是,少数无纤维蛋白原血症患者也可能患有严重的血栓栓塞并发症。这些患者的治疗特别具有挑战性,因为他们不仅有血栓形成的风险,还有出血的风险。我们报告一例33岁先天性无纤维蛋白原血症男性患者,该患者在两年内发生了两次心肌梗死。患者采用抗血小板治疗进行保守治疗,由于出血风险高未给予溶栓治疗。