Ozker Emre, Atalay Figen, Gulmez Oyku, Saritas Bulent
Department of Cardiovascular Surgery, Baskent University Istanbul Hospital, Oymaci Sk, No:7, Altunizade, 34662 Istanbul, Turkey.
Department of Hematology, Baskent University Istanbul Hospital, Istanbul, Turkey.
Indian J Hematol Blood Transfus. 2017 Sep;33(3):431-433. doi: 10.1007/s12288-016-0751-2. Epub 2016 Nov 21.
Congenital dysfibrinogenemia is a rare autosomal recessive bleeding disorder, which is characterized by the absence of functional fibrinogen. Patients may have bleeding and paradoxical arterial and venous thrombotic problems from early childhood. The optimal antithrombotic therapy in these patients hasn't been determined yet. In this report we present a dysfibrogenemic patient, who has suffered recurrent arterial thrombosis under aspirin treatment. Intravenous fibrinogen concentrates (fc) along with reduced doses of rivaroxaban (10 mg daily), cilostazol (50 mg bid) and aspirin (100 mg daily) were given as antithrombotic treatment. The pain and the cyanosis clinically recovered within 6 weeks. This is, to our knowledge, the first time in which a new oral anticoagulant, rivaroxaban and cilostazol combination was used in a dysfibrinogenemic patient with thrombotic episodes. We determined the type, the dosage and the duration of antithrombotic treatment according to the clinical progress of the symptoms. Rivaroxaban, cilostazol and fibrinogen concentrate replacement; combination may represent a useful alternative for the antithrombotic treatment in dysfibrinogenemic patients.
先天性纤维蛋白原异常血症是一种罕见的常染色体隐性出血性疾病，其特征是缺乏功能性纤维蛋白原。患者从幼儿期起可能就有出血以及矛盾性的动脉和静脉血栓形成问题。这些患者的最佳抗血栓治疗方案尚未确定。在本报告中，我们介绍了一名患有纤维蛋白原异常血症的患者，该患者在阿司匹林治疗期间反复发生动脉血栓形成。给予静脉注射纤维蛋白原浓缩剂（fc）以及减量的利伐沙班（每日10毫克）、西洛他唑（每日两次，每次50毫克）和阿司匹林（每日100毫克）进行抗血栓治疗。疼痛和发绀在6周内临床症状恢复。据我们所知，这是首次在一名患有血栓形成发作的纤维蛋白原异常血症患者中使用新型口服抗凝剂利伐沙班和西洛他唑联合治疗。我们根据症状的临床进展确定了抗血栓治疗的类型、剂量和持续时间。利伐沙班、西洛他唑和纤维蛋白原浓缩剂替代联合使用可能是纤维蛋白原异常血症患者抗血栓治疗的一种有用替代方案。
