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Abstract

The Antiphospholipid Syndrome (APS) is an acquired thrombophilic disorder in which auto antibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. APS is characterized by a hypercoagulable state potentially resulting in thrombosis of all segments of the vascular bed. Venous thrombosis typically presents with deep vein thrombosis (DVT) in the lower extremities. Other thrombotic presentations include osteonecrosis and venous occlusion of solid organs such as the liver (Budd-Chiari Syndrome (BCS)), kidneys and the adrenal glands with resulting in adrenal insufficiency. There are few reports about association between antiphospholipid antibodies (aPL) and development of BCS. We report the case of BCS development in young Russian male with primary APS. His disease began at the age of 15 with ileofemoral thrombosis of the left leg. Due to irregular taking of anticoagulants, the development of ileofemoral thrombosis on the right side was noted. An ascites and an evident hepatic insufficiency were noted after 5 years from the onset. In January 2011 an ascites gradually appeared and then because of the accumulation of large quantity of liquid, a laparocentesis was performed resulting in evacuation of 11 liters of liquid; gradual rising of liver failure was noted. Inferior vena cava thrombosis-a stenosis of its infrarenal part and BCS were diagnosed by CT angiography. BCS led to the development of liver cirrhosis with its evident functional deficiency and the development of multiple organ failure. An additional thrombosis risk factor was heterozygous prothrombin gene (G20210A) mutation. Besides that, the patient showed polymorphism in PAI-1 gene (4G/4G genotypes of PAI-1). The patient underwent orthotopic liver transplantation on 26.08.2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired.

Biography

Email: satybaldyeva_ma@rambler.ru