[Portal hypertension complicating Gaucher's disease (author's transl)]

On the occasion of a case of Gaucher's disease associated with portal hypertension and after reviewing similar cases in the literature, the following conclusions can be proposed: portal hypertension is a very rare complication of Gaucher's disease. An intrahepatic block or, more rarely, a constrictive pericarditis should be discussed. Splenectomy, which could be responsible for postoperative portal thrombosis, should be carried out only in patients with severe hypersplenism. In cases with proven portal hypertension, the indication for portacaval shunting should take into account the peculiar circumstances related to Gaucher's disease.