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A RARE PRESENTATION OF ANTIPHOSPHOLIPID SYNDROME: RECURRENT UPPER GASTROINTESTINAL BLEEDING SECONDARY TO PORTAL VEIN THROMBOSIS

Arindom Bora,Tribeni Sharma,Debdutta Gautam

2025 · DOI: 10.36106/ijsr/4803060
International journal of scientific research · 0 Citations

TLDR

In young patients without cirrhosis who present with variceal hemorrhage, APS should be considered an important differential diagnosis and early thrombophilia evaluation and timely initiation of anticoagulation are essential to improve outcomes and prevent further thrombotic complications.

Abstract

Background: Antiphospholipid syndrome (APS) is an autoimmune prothrombotic disorder characterized by recurrent venous or arterial thrombosis and the presence of antiphospholipid antibodies. While APS commonly manifests as deep venous thrombosis or cerebrovascular events, thrombosis of the portal venous system is rare, especially in the absence of cirrhosis. Portal vein thrombosis (PVT) can lead to non-cirrhotic portal hypertension, resulting in variceal bleeding, ascites, and hypersplenism. Early recognition of APS as an underlying cause of PVT is crucial for appropriate management and prevention of recurrent thrombotic events.

Case Presentation: We report the case of a 25-year-old female with recurrent upper gastrointestinal bleeding since adolescence, presenting with melena, hematemesis, and abdominal distension. She had undergone multiple endoscopic variceal ligation sessions across earlier admissions. On evaluation, she was hemodynamically unstable with anemia and thrombocytopenia. Imaging revealed a dilated portal vein with extensive periportal and perisplenic collaterals, splenomegaly, ascites, and portal cavernoma formation. Contrast-enhanced CT confirmed thrombosis of the branches of the portal vein. Thrombotic work-up demonstrated elevated anticardiolipin IgG (60.93 U/mL), consistent with APS, while ANA was negative. Upper GI endoscopy showed Grade II/III esophageal varices, for which EVL was performed. The patient was managed with non-selective β-blockers, rivaroxaban, transfusions, and was advised devascularization surgery due to recurrent variceal bleeding.

Conclusion: This case highlights a rare presentation of APS manifesting as non-cirrhotic portal vein thrombosis with recurrent upper gastrointestinal bleeding. In young patients without cirrhosis who present with variceal hemorrhage, APS should be considered an important differential diagnosis. Early thrombophilia evaluation and timely initiation of anticoagulation are essential to improve outcomes and prevent further thrombotic complications.