Antiphospholipid antibodies are related to portal vein thrombosis in patients with liver cirrhosis

The pathogenesis of portal vein thrombosis (PVT) in cirrhotic liver patients is not known. PVT has been related to liver dysfunction, neoplasm, hemodynamic factors, and hypercoagulability states. PVT has been reported in patients with antiphospholipid syndrome without liver cirrhosis. Our aim was to find the role of antiphospholipid antibodies (APAs) and coagulation inhibitors in PVT in patients with liver cirrhosis. We present a case-controlled study, matched by age, liver function, and etiology, to discover the role of APAs and anticoagulant protein activity in PVT in cirrhotic patients. We studied 30 cirrhotic patients: 6 of 10 (60%) patients with PVT were APA-positive, whereas only 2 of 20 (10%) in the cirrhotic control group were APA-positive (p < 0.005). Low serum levels of protein C, protein S antithrombin III, and plasminogen were found in cirrhotic patients; and, no differences were found between patients with and without PVT. Significantly lower protein S and antithrombin III levels were found in patients with Child-Pugh class C. Therefore, APAs were related to PVT in cirrhotic patients; but, a lower concentration of coagulation inhibitors was associated with liver dysfunction alone.     

Antiphospholipid syndrome presenting with inferior mesenteric vein thrombosis and associated rectal edema in a patient with systemic lupus erythematosus

Clinical Rheumatology -     

Congenital asplenia (Ivemark syndrome) revealed by mesenteric vein thrombosis in a 77 year old patient

Congenital asplenia (Ivemark syndrome) is usually associated with major cardiac malformations, which determine the clinical presentation and often result in death before 6 months of age. We report the unusual case of a 77 year-old patient with a congenital asplenia that was incidentally detected during a laparotomy for mesenteric vein thrombosis. The other abnormal findings in the abdomen were a para-esophageal hiatal hernia and left kidney hypotrophy. A segmental resection of the mid-jejunum was performed, with uneventful recovery. A thoraco-abdominal CT scan failed to reveal any associated vascular malformations in the retroperitoneum or the thorax. This case suggests that, in some instances, congenital asplenia, when isolated, may remain asymptomatic and be compatible with a long, and functionally normal life.     

Hydatid disease of the liver with portal vein invasion mimicking portal vein thrombosis

Hydatid cyst disease is a zoonosis caused by the parasite Echinococcus. It may infest any organ of the body, but it most frequently involves the liver, lungs, and nervous system. Portal vein involvement by hydatid cyst disease is extremely rare with only five cases published in the English literature to our knowledge. We present the ultrasonography (US) and computed tomography (CT) findings of a 77-year-old male with hydatid disease of the liver with portal vein invasion mimicking portal vein thrombosis. Colour Doppler US confirmed the lack of blood flow within the portal vein and stigmata of cavernomatosis. CT clearly demonstrated a communication between the multiloculated lesion and the portal vein and the multiple daughter vesicles obstructing the portal vein. The consideration of this complication will make it possible to distinguish this entity from portal vein thrombosis and, thus, the management of the patients with hydatid cyst disease particulary in endemic regions.     

Presinusoidal portal hypertension due to portal thrombosis in a patient with Alagille's syndrome

We present the case of a 16-year old woman with Alagille's syndrome, who had upper gastrointestinal bleeding due to rupture of esophageal varices secondary to presinusoidal portal hypertension without liver fibrosis. Portal thrombosis is a manifestation previously unreported in association to this syndrome.     

Antiphospholipid syndrome with acute myocardial infarction and portal vein occlusion: a case report

A 62-year-old woman was admitted to hospital because of chest oppression and abdominal discomfort. Coronary arteriography revealed that the proximal left anterior descending artery had a large thrombus with TIMI (Thrombolysis in Myocardial Infarction) Grade 3 flow. On the second hospital day, she had sudden hematemesis because of esophageal varices. Her general condition became stable with conventional therapy. On the 20th hospital day, coronary arteriography and arterial portography showed that the thrombus had diminished. Arterial portography also revealed total occlusion of the portal vein as well as giant gastric varices. She was diagnosed as antiphospholipid syndrome, based on the presence of lupus anticoagulant. The treatment of this case was very complicated because of the bleeding from the esophageal varices induced by the anticoagulant therapy for the thrombus. Prednisolone was administered for 1 month, but no remarkable effects were observed. Therefore, she was treated with endoscopic sclerotherapy for the esophageal varices and anticoagulant therapy for prevention of thrombosis.     

Primary antiphospholipid syndrome presented with portal vein thrombosis]

Antiphospholipid Antibody Syndrome (APS) is defined by arterial and venous thrombosis, recurrent spontaneous abortions and thrombocytopenia associated with persistence of antiphospholipid antibodies. Thrombosis may involve virtually all arterial or venous sites, but deep vein thrombosis of the lower limbs are the most common; however, unusual thrombi that involve the portal vein have been described. We report females with documented portal vein thrombosis and primary APS. The treatment of these patients is difficult because of the risk of bleeding and the recurrent thrombosis if they don't receive appropriate long-term anticoagulant therapy.     

Etiology and portal vein thrombosis in Budd-Chiari syndrome

AIM： To research the etiology, portal vein thrombosis and other features of Budd-Chiari syndrome （BCS） patients prospectively.
METHODS： A total of 75 patients （40 female, 35 male） who were diagnosed between January 2002 and July 2004 as having BCS were studied prospectively. Findings from on physical examination, ultrasonography, duplex ultrasonography and venography were analyzed. Hemogram and blood chemistry were studied at the time of diagnosis and on each hospital visit. Bone marrow examination and immune phenotyping were performed by a hematologist when necessary. Protein C, S, antithrombin Ⅲ, activated protein C resistance, and anticardiolipin antibodies, antinuclear antibodies, and anti ds-DNA were studied twice. The presence of ascite, esophageal varices, and portal thrombosis were evaluated at admission and on every visit.
RESULTS： At least one etiological factor was determined in 54 （72%） of the patients. The etiology could not be defined in 21 （28%） patients. One etiological factor was found in 39, 2 factors in 14 and 3 factors in 1 patient. The most common cause was the web （16%）, the second was Hydatid disease （11%）, the third was Behcet’s disease （9%）. Portal vein thrombosis was present in 11 patients and at least one etiology was identified in 9 of them （82%）.
CONCLUSION： Behcet’s disease and hydatid disease are more prominent etiological factors in Turkey than in other countries. Patients with web have an excellent response to treatment without signs of portal veinthrombosis while patients having thrombofilic factors more than one are prone to develop portal vein thrombosis with worse clinical outcome.     

A case of portal vein thrombosis in patient with ulcerative colitis

Portal vein thrombosis is a rare and serious complication in ulcerative colitis (UC). We report a patient with UC who developed portal vein thrombosis with persistent ascites which was successfully managed with total colectomy. A 46-year-old man was admitted complaining of bloody stool. UC had been diagnosed 11 years previously. He required subtotal colectomy because his colitis did not respond to conservative therapy and worsened with suspected peritonitis. Although the portal vein thrombosis was diagnosed after surgery and the systemic anti-coagulant therapy was started, this was stopped after 2 days because of massive rectal bleeding. Fortunately, sufficient hydration with intravenous infusion and re-infusion of concentrated ascites led to portal vein thrombolysis successfully after 28 postoperative days. This case suggests that colectomy and sufficient hydration may have a favorable effect on treatment of portal vein thrombosis in patients with UC.     

Portal and mesenteric vein thrombosis after portal vein embolization in a patient with protein S deficiency

Portal vein embolization can be performed safely, and so far no major complications have been reported. We report an extremely rare complication of portal vein embolization, a case of portal and mesenteric thrombosis in a 65-year-old patient with protein S deficiency. Right portal vein embolization was carried out prior to extended right hepatectomy for advanced gallbladder carcinoma involving the hepatic hilus. Computed tomography 14 days after embolization revealed massive thrombosis of the portal and the superior mesenteric veins. A protein S deficiency was found by means of an extensive workup for hypercoagulable state. Portal vein embolization may have triggered a cascade of events that was expressed as portal and mesenteric vein thrombosis resulting from deficiency of protein S. It may be better to determine the concentrations of such coagulation regulators prior to portal vein embolization.     

Sepsis and elevated liver enzymes in a patient with inflammatory bowel disease: think of portal vein thrombosis

A 42-year old man, 1 year previously diagnosed with ulcerative colitis after an emergency subtotal colectomy with formation of an ileostomy because of severe colitis with perforation, was admitted with sepsis and jaundice. The liver enzymes were elevated and blood cultures were positive for Streptococcus milleri. Magnetic resonance imaging showed a complete thrombosis of the main stem of the portal vein with occlusion of the left branch. Intravenous antibiotic therapy combined with heparinisation led to complete recanalisation of the thrombus. Portal vein thrombosis is a rare complication of inflammatory bowel disease and has been described in only 10 patients thus far. Multiple aetiologic factors may be responsible in relation to inflammatory bowel disease, such as hypercoagulability, thrombocytosis and abdominal sepsis. In patients with inflammatory bowel disease, unexplained sepsis and abnormal liver function tests, the possibility of an acute portal vein thrombosis should be considered and investigated, because unrecognised it may have serious long-term complications.     

Budd-Chiari syndrome, portal vein and mesenteric vein thrombosis in a patient homozygous for factor V Leiden mutation treated by TIPS and thrombolysis

We present the first case of Budd-Chiari syndrome in association with portal and mesenteric vein thrombosis in a patient homozygous for the factor V Leiden mutation. She was treated by transjugular intrahepatic portosystemic stent (TIPS) placement followed by local thrombolytic therapy. Venous outflow from the liver was established and the thrombi in the portal and mesenteric veins were lysed completely. This therapeutic approach may be used for such patients with this severe thrombotic event, who generally have a poor prognosis.     

终末期肝病合并门静脉血栓与肝移植术式研究

终末期肝病合并门静脉血栓(PVT)是指发生在门静脉主干、肠系膜上静脉、肠系膜下静脉或脾静脉的血栓。以往PVT被认为是肝移植的禁忌证,以后随着对PVT认识的不断提高和手术技术的逐步成熟,此类患者肝移植术效果也明显改善,PVT逐渐纳入肝移植的手术适应证。但终末期肝病合并PVT的肝移植术式多样,目前还没有统一术式,此文就其肝移植术式作一综述。