Introduction: Resistance to activated protein C is the most common inherited factor at the origin of deep venous thrombosis. As portal vein thrombosis is rare, causes such as cirrhosis, intra-abdominal infection, primary hepatocellular carcinoma, myeloproliferative disorders or coagulation abnormalities must be investigated.
Exegesis: We report two cases of portal vein thrombosis associated with resistance to activated protein C. This association is not frequent, as only 12 cases have been reported in the literature. These studies show that resistance to activated protein C was rarely the only factor, as other prothrombotic abnormalities were present in more than 70% of cases.
Conclusion: Resistance to activated protein C is rarely associated with portal vein thrombosis. When present, other causes should not be overlooked. The potential existence of resistance to activated protein C should be systematically investigated in case of either portal vein thrombosis in patients with personal or familial thrombosis history, association with multiple thrombosis, or when the disease etiology remains unknown.