Idiopathic portal hypertension

old Japanese woman was admitted to the hospital because of hematemesis.The clinical history was negative; she drank no alcohol and did not smoke.She did not recall episodes of jaundice and had not received needle sticks or transfusions.The patient had been well until she became nauseated after a meal and vomited some blood.On physical examination, she weighed 45 kg with a height of 148 cm.Her temperature was 36.7"C, her pulse was 70 beats per minute, her respiratory rate was 16 per minute and her blood pressure was 124/60 mm Hg.She had no rash or lymphadenopathy; stigmata of chronic liver disease such as spider angiomas or palmar erythema were absent.She was not jaundiced.The abdomen was soft, and the liver could not be felt.However, mild splenomegaly was noted.She had no peripheral edema.The remainder of the physical examination was negative.After a blood transfusion the hemoglobin level was 11.4 gm/dl, and the hematocrit was 32%; she had pancytopenia with 3,374,000 erythrocytes, 2,500 WBCs and 30,000 platelets.Levels of serum constituents were as follows: total bilirubin, 0.8 mgidl; alkaline phosphatase, 220 U/L (expected, 108 to 282); AST, 25 U/L (expected, less than 20); ALT, 15 U/L (expected, less than 24); total protein, 6.8 gnddl; total globulin, 2.7 gm/dl; and albumin, 3.6 gm/dl.The prothrombin time was normal (11.4 sec).Serological test results for the presence of antimitochondrial antibodies and for the detection of hepatitis B (core and surface) antibodies and antigens were negative.A test for hepatitis C was not available at the time.The urine was negative for protein and sugar.Endoscopy demonstrated esophageal and cardiac varices with cherry-red-spots.