原发性抗磷脂综合征合并布加综合征患者的肝移植
Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome.
作者信息
Reshetnyak Tatiana M, Seredavkina Natalia V, Satybaldyeva Maria A, Nasonov Evgeniy L, Reshetnyak Vasiliy I
机构信息
Tatiana M Reshetnyak, Natalia V Seredavkina, Maria A Satybaldyeva, Evgeniy L Nasonov, VA Nasonova Research Institute of Rheumatology, Kashirskoye Shosse, Moscow 115522, Russian Federation.
出版信息
World J Hepatol. 2015 Sep 8;7(19):2229-36. doi: 10.4254/wjh.v7.i19.2229.
The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of Budd-Chiari syndrome (BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.
抗磷脂综合征（APS）是一种获得性血栓形成倾向疾病，其中会产生针对细胞膜多种磷脂决定簇或磷脂结合蛋白的自身抗体。关于抗磷脂抗体与布加综合征（BCS）发生之间的关联报道较少。我们报告了一例患有原发性APS的年轻俄罗斯男性发生BCS的病例。该患者于2012年8月26日接受了原位肝移植。目前他的状况良好，肝脏血流恢复且功能未受损。我们首次报告了达比加群酯成功用于一名患有BCS的APS患者的长期抗凝治疗。此外，患者还接受免疫抑制药物治疗。
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