A 74-year-old female patient was admitted with diffuse abdominal pain, hyporexia and asthenia of 2 weeks' duration. She had been vomiting coffee grounds and melenic stools for 2 days. Her personal history included hypertension under treatment with amiloride/hydrochlorothiazide and dyslipidaemia controlled with lovastatin. She was a non-smoker and denied taking NSAIDs, alcohol or oral contraceptives. Her family history was: mother died of gastric cancer, brother with liver cirrhosis of unknown aetiology and sister with hepatocellular carcinoma.
- Physical examination: haemodynamic stability. Diffuse pain on abdominal palpation, with no signs of peritonism.
- Laboratory tests on admission: haemoglobin: 13.5 mg/dl; haematocrit: 37.6%; platelets: 120,000 platelets/ml; bilirubin: 247 mg/dl; alkaline phosphatase: 220 UI/l; aspartate aminotransferase: 59 UI/l; alanine aminotransferase: 28 UI/l and gamma-glutamyl transpeptidase: 129 UI/l. An emergency gastroscopy showed medium-sized oesophageal varices with no warning signs of imminent bleeding [F2 RC(-) according to the classification of the Japanese Society for the Study of Portal Hypertension] and multiple erosions with fibrin at gastric and duodenal level with no signs of recent haemostasis. On computed tomography and abdominal ultrasound, the liver was of normal size, with slightly undulating borders and a slightly heterogeneous parenchyma, with no space-occupying lesions. Splenomegaly, ascites and a small left pleural effusion were also observed. On the other hand, there was a complete non-occlusive portal thrombosis at the level of the hepatic hilum and its branches, as well as another thrombus at the level of the superior mesenteric vein with the presence of collateral circulation in the gastrohepatic and periesplenic ligaments. The aetiological study of the different causes of chronic liver disease included: hepatotropic viruses (HBV, HCV) and HIV, study of autoimmunity, ceruloplasmin, ferritin and porphyrins in urine, all results being negative. Subsequently, a percutaneous liver biopsy was performed with a haemodynamic study showing a portal pressure gradient of 12 mmHg with normal phlebography. The biopsy showed altered liver architecture due to hepatocyte hyperplasia in zone 1 of some of the acini, with a tendency to be nodular but without perinodular fibrosis septa. The portal tracts showed the usual duct-artery-vein triad, although the vein was sometimes inconspicuous and some paraportal vein was observed. They showed mild fibrous enlargement and contained a mild lymphoplasmacytic inflammatory infiltrate with focal overflow through the periportal interface. Some inflammatory foci were also observed in the lobule, although there were no acidophilic bodies or other relevant findings. These mild fibroinflammatory changes, the irregular nodular hyperplasia and the absence of perinodular fibrosis rule out the presence of cirrhosis and are compatible with non-cirrhotic portal hypertension. Given the possibility that all hepatic changes were secondary to primary portal thrombosis, a hypercoagulability study was performed.

This study showed an antigenic free protein S (pS), total antigenic pS and anticoagulant free pS of 56, 107 and 44% respectively, which was suggestive of a type I protein S deficiency. All other coagulation parameters (including fibrinogen, antithrombin, protein C, anticardiolipin antibodies, activated protein C resistance, factor V Leyden, factor II G 20210A and homocysteine) were in the normal range. This result was confirmed in a subsequent control.
The patient was placed on anticoagulant treatment with acenocoumarol and diuretics indefinitely. No new decompensations have been observed in the patient's follow-up.

