ABSTRACT

Of the several cases reviewed in 1994, the overwhelming number suffered from a "primary" Antiphospholipid Syndrome (APS), while only few had defined systemic lupus erythematosus. An active "endophlebitis" was demonstrated on liver biopsy which involved terminal hepatic veins, perhaps linking the condition to the changes seen in vessels in patients with the APS. Classic Budd-Chiari syndrome is a clinical and pathological entity, characterized by structural and functional abnormalities of the liver resulting from obstruction to the outflow of venous blood. Hepatic veno-occlusive disease, an unusual hepatic disorder, results in hepatomegaly and ascites, a presentation essentially similar to other more frequent causes of the Budd-Chiari syndrome. Splenic infarction usually does not seem to occur in an isolated manner but is associated with occlusions of other intraabdominal vessels, e.g., mesenteric or renal. The adrenal failure occurred simultaneously with pulmonary embolism and infarction in patients, and in a further several with pulmonary embolism independent of any preceding surgical procedure.