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Abstract
Portal vein thrombosis is an uncommon vascular disorder that may lead to portal hypertension and its complications. While cirrhosis and malignancy are the most frequent etiologies, inherited thrombophilias such as protein C and protein S deficiency are rare causes, especially in elderly patients.
PVT (Portal Vein Thrombosis) is a rare but clinically significant condition characterized by partial or complete obstruction of the portal venous system. It can present with nonspecific abdominal symptoms or features of portal hypertension, making early diagnosis challenging. The condition is most commonly associated with liver cirrhosis, hepatobiliary malignancies, and intra-abdominal infections.[1] However, in a subset of patients without these risk factors, an underlying hypercoagulable state must be considered.
Inherited deficiencies of natural anticoagulants such as protein C and protein S are well-recognized causes of venous thromboembolism, typically manifesting as deep vein thrombosis or pulmonary embolism at a younger age. Their association with isolated portal vein thrombosis in elderly individuals is rare and often underdiagnosed. This case report describes a rare presentation of extensive portal vein thrombosis secondary to protein C and protein S deficiency in a non-cirrhotic elderly patient, emphasizing the need for thorough etiological evaluation.
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References
- Amitrano L, Guardascione MA, Brancaccio V, et al. Risk factors and clinical presentation of portal vein thrombosis in cirrhosis. J Hepatol 2004;40(5):736-41.
- Boccatonda A, Di Stadio F, Cocco G, et al. Portal vein thrombosis: state-of-the-art review. J Clin Med 2021;10(3):581.
- DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology 2009;49(5):1729-64.
- European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol 2016;64(1):179-202.
- Kumar S, Sarr MG, Kamath PS. Mesenteric venous thrombosis. N Engl J Med 2001;345(23):1683-8.
- Janssen HL, Meinardi JR, Vleggaar FP, et al. Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors in Budd–Chiari syndrome and portal vein thrombosis. Blood 2000;96(7):2364-8.
- Denninger MH, Chaït Y, Casadevall N, et al. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 2000;31(3):587-91.
- Condat B, Pessione F, Hillaire S, et al. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 2001;120(2):490-7.
- Primignani M, Barosi G, Bergamaschi G, et al. Natural history of portal vein thrombosis in adults without liver disease: a multicenter longitudinal study. Blood 2005;105(12):4736-41.
- Darwish Murad S, Valla DC, de Groen PC, et al. Determinants of survival and effect of portosystemic shunting in patients with Budd–Chiari syndrome. Hepatology 2004;39(2):500-8.
References
Amitrano L, Guardascione MA, Brancaccio V, et al. Risk factors and clinical presentation of portal vein thrombosis in cirrhosis. J Hepatol 2004;40(5):736-41.
Boccatonda A, Di Stadio F, Cocco G, et al. Portal vein thrombosis: state-of-the-art review. J Clin Med 2021;10(3):581.
DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology 2009;49(5):1729-64.
European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol 2016;64(1):179-202.
Kumar S, Sarr MG, Kamath PS. Mesenteric venous thrombosis. N Engl J Med 2001;345(23):1683-8.
Janssen HL, Meinardi JR, Vleggaar FP, et al. Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors in Budd–Chiari syndrome and portal vein thrombosis. Blood 2000;96(7):2364-8.
Denninger MH, Chaït Y, Casadevall N, et al. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 2000;31(3):587-91.
Condat B, Pessione F, Hillaire S, et al. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 2001;120(2):490-7.
Primignani M, Barosi G, Bergamaschi G, et al. Natural history of portal vein thrombosis in adults without liver disease: a multicenter longitudinal study. Blood 2005;105(12):4736-41.
Darwish Murad S, Valla DC, de Groen PC, et al. Determinants of survival and effect of portosystemic shunting in patients with Budd–Chiari syndrome. Hepatology 2004;39(2):500-8.