Inherited antithrombin III deficiency causing mesenteric venous infarction: a new clinical entity

Primary superior mesenteric venous thrombosis is sometimes preceded by peripheral thrombophlebitis. Inherited antithrombin III deficiency is a recently recognized autosomal dominant trait, which is characterized by thrombophlebitis and pulmonary embolism. This case report illustrates many features of both entities and strongly suggests a causal relationship. While longterm therapy has yet to be established, prophylactic therapy is recommended when asymptomatic individuals with known antithrombin III deficiency are at increased risk of thrombosis. The efficacy of heparin alone has been unreliable, whereas Coumadin has been encouraging. Antithrombin III concentrates are being developed and theoretically should be helpful. Patients with thrombophlebitis or pulmonary embolism should be suspected of having antithrombin III deficiency. Such individuals also represent one mechanism to explain 'primary' mesenteric venous thrombosis.