Gaucher's disease and portal hypertension

Abstract

Two cases of Gaucher's disease with portal hypertension are dscribed, of which one was relieved by splenectomy and the other, which had recurrent bleeding from oesophageal varices, by portacaval anastomosis. These cases demonstrate that portal hypertension in this condition may be due to increased forward portal blood-flow due to splenomegaly, to intrahepatic obstruction from extensive deposits of Gaucher cells, and probably in some cases to a combination of these factors. The case with bleeding oesophageal varices was also infantile and neither of these conditions has previously been described in Gaucher's disease.