Related Experiment Videos
Deficiency of protein C in patients with portal vein thrombosis
H Orozco1, E Guraieb, T Takahashi
1Portal Hypertension Clinic, Instituto Nacional de la Nutricion Salvador Zubiran, Mexico City, Mexico.
Insights
Protein C deficiency is linked to portal vein thrombosis (PVT) and bleeding varices. Investigating this deficiency is crucial for patients with PVT and a history of blood clots.
Area of Science:
- Hepatology
- Hematology
- Vascular Medicine
Background:
- Portal vein thrombosis (PVT) is often idiopathic in adults.
- Protein C deficiency is a hereditary condition predisposing individuals to thromboembolic events.
Observation:
- Two patients presented with portal hypertension and bleeding varices secondary to PVT.
- Both patients had a history of recurrent systemic thromboembolic disease and mesenteric venous thrombosis.
Findings:
- Hematologic work-up confirmed Protein C deficiency in both patients.
- Liver biopsies were normal, indicating the deficiency was the primary cause.
- Surgical intervention (Sugiura procedure) followed by anticoagulation led to positive outcomes.
Implications:
- Protein C deficiency should be screened in all PVT cases, particularly those with a history of thromboembolism.
- Early diagnosis and management of Protein C deficiency can improve outcomes for PVT patients.
- This highlights the importance of a comprehensive hematologic evaluation in unexplained PVT.
Abstract:
Portal vein thrombosis has been considered idiopathic in 50% of cases reported in adults. Protein C deficiency is a recently described disorder characterized by a predisposition to develop thromboembolic disease. We report the findings in two patients with portal hypertension and bleeding varices due to portal vein thrombosis in whom a deficiency of protein C was present. Both cases were very similar, with a history of recurrent episodes of systemic thromboembolic disease, mesenteric venous thrombosis that required intestinal resection and upper gastrointestinal bleeding from gastroesophageal varices. Portal hypertension as well as portal vein thrombosis were demonstrated. The hematologic work-up revealed a deficiency of protein C. Both patients were subjected to the Sugiura procedure, and anticoagulation was instituted thereafter. At the time of surgery, a liver biopsy was performed, which was reported as "normal." Two years and 3 months, respectively, after surgery both patients are in good condition. We conclude that protein C deficiency should be investigated in all cases of portal vein thrombosis, especially in those with a history of thromboembolic disease elsewhere.