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Published on: June 27, 2025
[Portal and mesenteric thrombosis associated with protein S deficiency]
J A Chirinos Vega1, R Muñoz Gómez, M Amo Peláez
1Departamento de Medicina de Aparato Digestivo. Hospital Universitario 12 de Octubre. Madrid.
Portal thrombosis (PT) can stem from rare hypercoagulability syndromes like protein S deficiency, not just liver cirrhosis. Early diagnosis and anticoagulant treatment are crucial for preventing further thrombotic events.
Area of Science:
- Hepatology
- Hematology
- Vascular Medicine
Background:
- Liver cirrhosis is the primary cause of portal thrombosis (PT).
- Hypercoagulability syndromes are infrequently identified as the etiology of PT.
- This case highlights a rare instance of PT secondary to protein S deficiency.
Observation:
- A 74-year-old woman presented with melena and abdominal pain, revealing ruptured esophageal varices.
- Imaging demonstrated extensive portal and mesenteric thrombosis, splenomegaly, ascites, and portosystemic collaterals.
- Liver biopsy suggested non-cirrhotic portal hypertension, prompting further investigation for hypercoagulability.
Findings:
- Protein S deficiency was diagnosed, with total pS at 107% and free pS at 56%.
- The patient received anticoagulant therapy, which successfully prevented additional thrombotic events.
- This case underscores the importance of investigating hypercoagulability in PT patients.
Implications:
- PT diagnosis necessitates a thorough evaluation for underlying hypercoagulability disorders.
- Protein S deficiency should be considered in the differential diagnosis of PT, even with suspected liver disease.
- Systematic screening for prothrombotic states improves patient outcomes in portal thrombosis management.
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