- Characterized by sudden formation of a thrombus within the portal vein
- symptoms: sometimes presents with sudden onset RUQ or lumbar pain, symptoms can be absent
- systemic (70% of cases): myeloproliferative disease, OCP use, inherited thrombophilias, paroxysmal nocturnal hemoglobinuria
- local (30% of cases): inflammatory disease (e.g.. pancreatitis, IBD, cholangitis), infectious diseases (abdominal sepsis), surgery, trauma, cirrhosis, malignancies
- presence of fevers/abdominal pain is concerning for acute pylephlebitis
- anticoagulation beneficial to increase recanalization rates and prevent complication of intestinal infarction (as opposed to unclear indication for anticoagulation with chronic portal vein thrombosis)
- portal vein thrombosis is frequently the first manifestation of a chronic myeloproliferative disease (CMPD) (identified in 25-30% of patients); it can be difficult to diagnose given acute alterations in the blood cell counts during acute thrombosis and the finding that many patients present with a masked or atypical myeloproliferative disorder
- Jak2 V617F mutation is highly specific diagnostic marker of CMPD (because it is not detected in healthy individuals), although it can be absent in 20-60% of patients with chronic myeloproliferative disorders
Spaander: Portal Vein Thombosis in non cirrhotic patients. Optima Grafische Communicatie. Dec 2010.
Primignani M, Barosi G, Bergamaschi G, Gianelli U, Fabris F, Reati R, et al. Role of the JAK2 mutation
in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology.
2006 Dec; 44(6): 1528‑34.