Background: Isolated sinusoidal dilatation is an uncommon hepatic lesion and t he cause is largely unknown. Objective: To investigate whether prothrombotic dis orders or perisinusoidal cell changes could be involved i...Background: Isolated sinusoidal dilatation is an uncommon hepatic lesion and t he cause is largely unknown. Objective: To investigate whether prothrombotic dis orders or perisinusoidal cell changes could be involved in pure idiopathic hepat ic sinusoidal dilatation (HSD). Methods: Evaluation for associated conditions, p rothrombotic disorders, and studies of hepatic perisinusoidal cell activation in consecutive patients, seen between 1993 and 2002, with isolated sinusoidal dila tation unrelated to outflow block, sinusoidal infiltration, or hepatic granuloma s. Results: Among 11 patients, associated conditions were prothrombotic disorder s (n = 5) and oral contraceptive use (n = 3). Prothrombotic disorders were polyc ythemia vera (n = 1) and anticardiolipin antibodies combined with lupus anticoag ulant (n = 4). No genetic thrombophilia factor was found. Of four patients with lupus anticoagulant, three had antinuclear factors and high serum levels of anti cardiolipin antibodies at repeated testing. There was no evidence of intrahepati c or extrahepatic thrombosis in any of the patients. Sinusoidal dilatation was m arked in six of 11 patients (54%), including two patients with antiphospholipid antibodies. Activated perisinusoidal cells were only found around markedly dila ted sinusoids. Conclusion: Idiopathic pure HSD is frequently associated with the immunological features of the antiphospholipid syndrome. Therefore, finding pur e HSD in a liver biopsy specimen should prompt the search for antiphospholipid a ntibodies.展开更多
文摘Background: Isolated sinusoidal dilatation is an uncommon hepatic lesion and t he cause is largely unknown. Objective: To investigate whether prothrombotic dis orders or perisinusoidal cell changes could be involved in pure idiopathic hepat ic sinusoidal dilatation (HSD). Methods: Evaluation for associated conditions, p rothrombotic disorders, and studies of hepatic perisinusoidal cell activation in consecutive patients, seen between 1993 and 2002, with isolated sinusoidal dila tation unrelated to outflow block, sinusoidal infiltration, or hepatic granuloma s. Results: Among 11 patients, associated conditions were prothrombotic disorder s (n = 5) and oral contraceptive use (n = 3). Prothrombotic disorders were polyc ythemia vera (n = 1) and anticardiolipin antibodies combined with lupus anticoag ulant (n = 4). No genetic thrombophilia factor was found. Of four patients with lupus anticoagulant, three had antinuclear factors and high serum levels of anti cardiolipin antibodies at repeated testing. There was no evidence of intrahepati c or extrahepatic thrombosis in any of the patients. Sinusoidal dilatation was m arked in six of 11 patients (54%), including two patients with antiphospholipid antibodies. Activated perisinusoidal cells were only found around markedly dila ted sinusoids. Conclusion: Idiopathic pure HSD is frequently associated with the immunological features of the antiphospholipid syndrome. Therefore, finding pur e HSD in a liver biopsy specimen should prompt the search for antiphospholipid a ntibodies.
