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BACKGROUND AND AIM Comprehensive investigations on the prothrombotic factors of splanchnic vein thrombosis (SVT), including Budd-Chiari syndrome (BCS) and non-cirrhotic nonmalignant portal vein thrombosis (PVT), in Eastern patients are scarce. METHODS Between March 2012 and July 2017, 812 consecutive patients, including 418 BCS and 394 non-cirrhotic nonmalignant PVT patients, were admitted to Xijing Hospital (a Chinese tertiary academic hospital) and screened for prothrombotic factors. Odds ratios (ORs), 95% confidence intervals (CIs), and P-trends were calculated by using conditional logistic regression. RESULTS The prevalence of myeloproliferative neoplasms (MPNs) was only 6.3% among BCS patients but 28.3% among PVT patients. Notably, the presence of MPNs was associated with a higher risk of hepatic vein-type BCS (OR 9.9, 95% CI 3.6-26.7, P-trend

The Budd-Chiari syndrome is caused by obstruction of blood flow in hepatic veins. The site of obstruction can be small or larger hepatic veins or the supra-hepatic portion of the inferior vena cava (IVC). Hepatic vein thrombosis predominates in western countries while supra-hepatic IVC obstruction or the combined effects of hepatic vein and IVC obstruction is more common in China and other parts of Asia. In this report, we describe a patient with the Budd-Chiari syndrome who had obstruction of a long segment of infra-hepatic IVC. The clinical features in this patient were restricted to prominent veins on his abdominal and chest walls. In 2010, a 23-year-old man was investigated in our department because of prominent blood vessels on his abdominal wall that had been increasing in size for approximately 12 months. There was no previous history of liver disease or alcohol abuse. The appearance of veins on his abdominal and chest walls are shown in Figure 1, left. Blood tests revealed a normal hemoglobin, normal liver and renal function tests and normal coagulation studies. Serological tests for hepatitis B and C were negative. A color Doppler ultrasound study showed obstruction of the middle and right hepatic veins but a patent left hepatic vein. An abdominal computed tomography (CT) scan showed that the supra-hepatic portion of the IVC was patent (Figure 1, right). However, the infra-hepatic portion of the IVC was extensively obstructed as shown in the axial (Figure 2, left) and coronal images (Figure 2, right). The CT scan also showed dilated azygos and semi-azygos veins (Figure 1, right, white solid arrows) and tortuous veins on the abdominal wall (Figure 1 right and Figure 2 left, interrupted arrows). He did not have splenomegaly or ascites and there were no features of esophageal or gastric varices at upper gastrointestinal endoscopy. An attempt was made to outline the IVC via the femoral veins but both the right and left iliac veins were obstructed. Hepatic vein angiography via the right internal jugular vein showed that a dilated accessory hepatic vein was patent. The diagnosis was that of a Budd-Chiari syndrome with obstruction of a long segment of the infra-hepatic IVC. He has been treated with long-term anticoagulants. Screening tests for anticardiolipin antibodies and various pro-thrombotic mutations were negative. However, he did have an elevated level of homocysteine (31.26 mmol/l) and a low level of vitamin B12 (95 pg/ml). His clinical status has been unchanged after follow-up for 2 years.

In cirrhosis, the development of hepatic encephalopathy is largely determined by the function of residual hepatocytes and by the extent of vascular collaterals that divert portal blood to the systemic circulation. These portosystemic shunts can be categorized into intrahepatic and extrahepatic types. Common extrahepatic shunts involve gastroesophageal, gastrorenal, splenorenal and paraumbilical vessels. Under most circumstances, the presence of hepatic encephalopathy is a relative contraindication to the insertion of a transjugular intrahepatic portosystemic shunt (TIPS). However, in the patient described below, TIPS permitted closure of an unusual shunt that was followed by improvement in encephalopathy. In 2011, a woman, aged 47, was admitted to our hospital with episodes of drowsiness attributed to hepatic encephalopathy. She was known to have hepatitis B with advanced cirrhosis (ChildPugh class C). In 2006, she was treated medically for a major gastrointestinal bleed attributed to esophageal varices but there were no subsequent episodes of bleeding. Medical treatment for hepatic encephalopathy had included dietary protein restriction, lactulose, branched-chain amino acids and a course of rifaximin. On examination, she had several spider nevi and peripheral edema. An abdominal ultrasound study and a contrast-enhanced computed tomography scan showed that the portal vein was relatively narrow. However, the splenic vein, inferior mesenteric vein, left renal vein and left gonadal vein were markedly dilated. Subsequently, a catheter was passed into the left renal vein through the inferior vena cava. The injection of contrast revealed dilatation of the left renal vein and left gonadal vein and a varicose shunt between the left gonadal vein and the left inferior mesenteric vein. A transjugular intrahepatic portosystemic shunt was then placed within the liver to reduce the portal pressure. Direct portography at the time of insertion of the stent (Figure 1) shows the stent (black arrowhead), the splenic vein (white arrowhead), the inferior mesenteric vein (solid arrow) and the varicosity (dotted arrow) linking the inferior mesenteric vein to the left gonadal vein. The shunt was successfully embolized using coils (Figure 2). The patient has now been followed for 4 months without a recurrence of encephalopathy and without dietary or drug therapy. Patency of the portosystemic shunt was confirmed by an ultrasound study.

Hepatosplenic gamma-delta (gd) T-cell lymphoma is a rare form of peripheral T-cell lymphoma that was first proposed as a distinct clinicopathologic entity in 1990. Common presenting symptoms include fever, weakness and abdominal pain. On examination, the liver and spleen are enlarged but there are no enlarged peripheral lymph nodes. Typical blood tests reveal anemia, leukopenia and thrombocytopenia but liver function tests are either normal or near-normal. Histological features are characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen and sinuses of the bone marrow. Immunophenotypic expressions include CD2 + , CD3 + , CD4 , CD5, CD7, CD16, CD56, TIA-1 + , TdT and granzyme B. The T-cell receptor—gd phenotype (TCRd1 + ) is positive while the T-cell receptor—ab phenotype (bF1) is negative. Thus far, responses to chemotherapeutic regimens have been poor and patients have a median survival of only 11‐16 months. Of interest to gastroenterologists are case reports of hepatosplenic gd T-cell lymphomas in patients with inflammatory bowel disease after treatment with monoclonal antibodies against tumor necrosis factor. Most of these patients have been young men receiving concomitant medication with azathioprine, 6-mercaptopurine or methotrexate. The patient illustrated below was a 46-year-old man who was admitted to hospital with upper abdominal distension and peripheral edema. Twelve months previously, he had been treated by splenectomy for splenomegaly and features of hypersplenism. An abdominal computed tomography scan showed a huge liver that extended into the pelvis. The liver had a heterogeneous appearance but no discrete nodules (Figure 1). Blood tests revealed mild anemia (hemoglobin 108 g/l), a mild elevation of the white cell count (13.4x10 9