Idiopathic portal hypertension in a systemic sclerosis patient heterozygous for factor V Leiden mutation.

Abstract excerpt

Here we present a rare case of systemic sclerosis (SSc) with idiopathic portal hypertension (IPH) having factor V Leiden mutation, a well-known genetic risk factor for various venous thromboses. A 53-year-old SSc patient showing huge esophageal varices and splenomegaly without liver cirrhosis was diagnosed with IPH. As heterozygous Leiden mutation was detected, some coagulation abnormality and resultant formation...

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