W e describe four patients with hepatic vein thrombosis caused by Behqet's disease and review the 17 previously published cases. In addition, we compared these 21 cases of hepatic vein thrombosis to our 24 cases of hepatic vein thrombosis caused by primary myeloproliferative disorders. In patients with Behpet's disease, a male predominance (male/female ratio, 19 : 1) contrasted with the female predominance found in patients with hepatic vein thrombosis complicating primary myeloproliferative disorders (sex ratio = 1 : 3). The mean age at clinical onset was younger in patients with Behqet's disease than in those with primary myeloproliferative disorders (29 vs. 36 yr). Obstruction of the inferior vena cava was found in 90% of patients with hepatic vein thrombosis caused by Behget's disease. Inferior vena caval thrombosis appears to be the main pathophysiological mechanism of hepatic vein thrombosis in patients with Behqet's disease. Unlike patients with primary myeloproliferative disorders who often had a progressive course, one third of patients with Behqet's disease had acute liver failure and died within 2 wk of clinical onset. These findings suggest that, in patients with Behqet's disease, hepatic vein thrombosis is a sudden event usually related to the extension of a caval thrombus to the ostium of the hepatic veins. (HEPATOLOGY 1990;11:969-974.) Originally described as a clinical triad of oral and genital ulcerations with iridocyclitis (11, Behget's disease is now recognized as a multisystemic disorder (2, 3). Vasculitis is thought to be the mechanism common to most Behset's disease manifestations (4). Venous occlusion is frequent (2, 3, 5, 61, but involvement of the hepatic veins is uncommon.
The purpose of this report is (a) to describe four patients with hepatic vein thrombosis (HVT) caused by Behget's disease, (b) to review the 17 previously published cases of HVT caused by Behget's disease and (c) to compare these 21 cases of HVT to 24 personal cases of HVT caused by primary myeloproliferative disorders, which are the main causes of HVT.