Nodular regenerative hyperplasia of the liver: report of two cases and review of the literature.

We review the clinical and pathologic features of 39 cases of nodular regenerative hyperplasia of the liver and include two new cases. This is an uncommon but distinct pathological entity characterized by regenerative hepatocytic nodules without fibrosis. Portal hypertension that often leads to hemorrhage from esophageal varices is the most important clinical manifestation. Characteristically, manifestations of hepatic failure are lacking, but a number of patients have elevated alkaline phosphatase of hepatic origin. Felty's syndrome is the entity most often associated with nodular regenerative hyperplasia. Anemia, leukopenia, and thrombocytopenia are frequent hematologic abnormalities secondary to hypersplenism. The etiology and pathogenesis of nodular regenerative hyperplasia of the liver are unknown.