Nonmalignant portal vein thrombosis in adults

Abstract

Portal vein thrombosis (PVT) consists of two different entities: acute PVT and chronic PVT. Acute PVT usually presents as abdominal pain. When the thrombus extends to the mesenteric venous arches, intestinal infarction can occur. Chronic PVT is usually recognized after a fortuitous diagnosis of hypersplenism or portal hypertension, or when there are biliary symptoms related to portal cholangiopathy. Local risk factors for PVT, such as an abdominal inflammatory focus, can be identified in 30% of patients with acute PVT; 70% of patients with acute and chronic PVT have a general risk factor for PVT, most commonly myeloproliferative disease. Early initiation of anticoagulation therapy for acute PVT is associated with complete and partial success in 50% and 40% of patients, respectively. A minimum of 6 months' anticoagulation therapy is recommended for the treatment of acute PVT. For patients with either form of PVT, permanent anticoagulation therapy should be considered if they have a permanent risk factor. In patients with large varices, beta-adrenergic blockade or endoscopic therapy seems to prevent bleeding as a result of portal hypertension, even in patients on anticoagulation therapy. In patients with jaundice or recurrent biliary symptoms caused by cholangiopathy, insertion of a biliary endoprosthesis is the first treatment option. Overall, the long-term outcome for patients with PVT is good, but is jeopardized by cholangiopathy and transformation of underlying myeloproliferative disease into myelofibrosis or acute leukemia.

Interventional treatment for symptomatic acute-subacute portal and superior mesenteric vein thrombosis

“…If the following conditions occur, the possibility of PV-SMV thrombosis should be considered in order to achieve early diagnosis and treatment. largement without apparent splenic hyperfunction; (6) unexplained paralytic intestinal obstruction, necrosis or peritonitis, etc [8,9] . The diagnosis of PV-SMV thrombosis relies on imaging.…”

Portal Vein Thrombosis

“…Occult MPD as a cause of PVT is seen in 16.7% and classical MPD in 13.8%. 18 The diagnosis of myeloproliferative disorders as a cause of PVT has increased by 20% with the identification of Janus kinase 2 (JAK 2) V617F gene mutation. The presence of JAK 2 mutation is seen in around 17-35% patients of PVT.…”

“…Prothrombin levels and other coagulation factors may be decreased while Ddimer is usually increased. 18,84 Patients with portal hypertensive biliopathy may show a rise in alkaline phosphatase. Liver grossly is normal in PVT, but may show atrophy and regenerative nodular hyperplasia, related to apoptosis and compensatory arterial vasodilation in chronic PVT.…”

Portal vein thrombosis – a primer for the general physician

“…23 One study showed the figure to be 30.7%, with 16.7% being occult MPD and 13.8% being classical MPD. 24 Another recent study, looking at 44 patients, reported PVT to be the first manifestation of MPD in 31 patients (70%). 25 However, this mutation is not very common in those with PVT secondary to liver disease.…”

“…As is generally the case in venous thrombosis, D-dimer is usually increased and not helpful. 24 It is important to exclude an underlying systemic disorder in those with no local cause identified. All patients without a clearly identifiable cause on imaging should have a JAK2 mutation analysis, lactate dehydrogenase and tests for paroxysmal nocturnal haemoglobinuria , factor V Leiden and prothrombin gene mutations.…”