Author(s): Marrone E*, Mastrobuoni C, Abate A, D’Auria D, Cinque F, Magliocca A, Valentino U, Morella P and Tufano A
Background
Inherited defects of the natural coagulation inhibitors predispose patients to thrombosis. These disorders have similar clinical presentations with a strong family of thrombosis, episodes of recurrent venous thromboembolism, beginning in early adulthood. We report a case of portal vein thrombosis (VPT) secondary to hereditary protein C deficiency in a young male.
A 39-year-old male was admitted to the hospital for abdominal pain. Laboratory tests revealed levels of alanine and aspartate aminotransferases increased and levels of lipase and amylase normal, ruling out any possibility of pancreatitis. Abdominal CT scan indicated thrombosis of the portal vein and multiple celiac lymph nodes. To exclude occult malignancy PET/CT scan was conducted. Results from viral profiles and tumor markers were negative. Thrombophilic screening was negative except for low levels of protein C (PC) antigen (28% with n.r. 70-140%). Therefore, insufficient PC came out to be the primary cause of PVT. The patient was prescribed anticoagulant therapy with fondaparinux 7,5 mg/die and then warfarin for a long period to target an INR range of 2-3.
Inherited PC deficiency is rare in PVT. Its identification is important for treatment of PVT, with better outcomes associated with early anticoagulant medication intervention.
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