Background Portal vein thrombosis (PVT) is a rare and severe clinical phenotype of antiphospholipid syndrome (APS) with a poor prognosis. Anticoagulation therapy is efficient, but is associated with potentially severe side-effects, especially bleeding episodes. The aim of this study was to retrospectively analyze our single center experience on long-term anticoagulation in APS patients presenting a PVT.
Methods A retrospective study of APS patients with PVT from 2012 to 2019 was conducted using the Hospital Information System of Peking Union Medical College Hospital. Basic clinical history and complications were collected. Regular imaging was performed to monitor the outcome of PVT. The recanalization rate of the PVT after anticoagulation was analyzed using the survival analysis.
Results A total of 28 patients with APS-PVT were enrolled, 5 males and 23 females, with the median age 37 years (range 17–63 years), and the mean follow-up was 3 years (range, 0.5–7 years). 8 cases were acute thrombosis, 16 cases chronic thrombosis, and 4 cases portal vein cavernoma. The first symptoms presented as abdominal distention (14/28) or pain (7/28) and blood system involvements (22/28, anemia or thrombocytopenia), while presentation with variceal bleeding (4 cases) was less common, and 2 patients were asymptomatic. Triple aPLs positive in 7 cases. 10 cases began efficient anticoagulation therapy immediately at the diagnosis of thrombus. 8 patients got thrombus recanalization. 3 patients got recurrence. 5 patients died. Survival analysis revealed that effective anticoagulation could increase recanalization rate significantly (log rank p =0.001), as shown in figure 1.
Conclusions PVT usually had insidious onset with atypical clinical symptoms and easily be misdiagnosed. Early diagnosis and efficient anticoagulation treatment can bring thrombus recanalization thereby significantly improving the prognosis.
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