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.     

Protein C and D-dimer are related to portal vein thrombosis in patients with liver cirrhosis

Background and Aim: To profile changes of coagulation, anticoagulation and fibrolytic factors associated with liver function failure and portal vein thrombosis (PVT) formation in chronic liver cirrhosis patients. Methods: A total of 116 cirrhotic patients admitted to our hospital from June 2006 to October 2008 were included in our study. All patients were classified into two groups: PVT group (31 patients), composed of patients with PVT and a control group (85 patients), including patients without PVT. Platelet, prothrombin time (PT), activated partial prothrombin time (APTT) and fibrinogen were measured. Also, plasma samples from the patients were analyzed for the levels of antithrombin III (AT‐III), protein C (PC), protein S (PS), D‐dimer, tissue‐type plasminogen activator as well as plasminogen activator inhibitor‐1. Statistical analyses were carried out to evaluate the correlation of specific variations with the disease status. Results: In general, the higher Child‐Pugh scores, indicating the aggravation of hepatic impairment of the patients, correlated well with the prolonged PT/APTT and increased D‐dimer, as well as decreased platelet, fibrinogen, PC and AT‐III levels in the serum. Furthermore, we found that the PC, PS and D‐dimer levels in PVT patients were 2.32 ± 0.72 mg/L, 17.14 ± 3.62 mg/L and 0.99 ± 0.36 mg/L, respectively, both representing a significant difference compared with those in the control group without PVT. Logistic regression model shows that the odds ratio value of one unit of increase of PC and D‐dimer were 0.48 and 15.57. Conclusions: Cirrhotic patients displayed dysfunctions in the coagulation, anti‐coagulation and fibrolytic systems. The development of PVT in these patients may be independently associated with the decrease of PC, PS and D‐dimer. Furthermore, decreasing PC and increasing D‐dimer may be risk factors inducing PVT in cirrhotic patients.     

Mesenteric venous thrombosis associated with protein C deficiency

An 83-year-old man had gradually worsening abdominal pain and vomiting. Laparotomy revealed segmental intestinal infarction resulting from thrombosis in the superior mesenteric vein. Necrosed intestine was resected and anastomosis was performed successfully. The patient was anticoagulated with intravenous heparin and nafamostat mesilate followed by oral aspirin. He recovered rapidly. Blood chemistry revealed protein C deficiency, while protein S and antithrombin III levels were normal. Laboratory evaluation of these proteins may help define the cause of mesenteric venous thrombosis. Received: August 14, 1998/Accepted: December 18, 1998     

Extensive mesenteric vein and portal vein thrombosis successfully treated by thrombolysis and anticoagulation

Mesenteric vein thrombosis is generally difficult to diagnose and can be fatal. A case of extensive thrombosis of the mesenteric and portal veins was diagnosed early and successfully treated in a 26-year-old man with Down syndrome who was admitted to hospital because of abdominal pain, severe nausea and high fever. Ultrasonography revealed moderate ascites, and there was minimal flow in the portal vein (PV) on the Doppler examination. Computed tomography (CT) showed remarkable thickening of the walls of the small intestine and extensive thrombosis of the mesenteric, portal and splenic veins. Because neither intestinal infarction nor peritonitis was seen, combined thrombolysis and anticoagulation therapy without surgical treatment was chosen. Urokinase was administered intravenously and later through a catheter in the superior mesenteric artery. Heparin and antibiotics were given concomitantly. The patient's symptoms and clinical data improved gradually. After 10 days, CT revealed that collateral veins had developed and the thrombi in the distal portions of the mesenteric veins had dissolved, although the main trunk of the PV had not recanalized. The only risk factor of thrombosis that was detected was decreased protein S activity.     

急性广泛门静脉系统血栓合并肺栓塞1例报告

门静脉血栓形成（portal vein thrombosis,PVT）是由于门静脉血管腔内血栓形成造成门静脉内血流部分或完全受阻的病理生理过程,人群中发病率为0.6%～1%[1],分为急性和慢性血栓[2]。急性门静脉血栓形成是一种临床表现复杂且少见的急腹症,占肠道血管性疾病的5%～15%[3]。而急性广泛门静脉系统血栓形成是指门静脉、脾静脉、肠系膜上静脉、肠系膜下静脉中有2条或2条以上血管急性血栓形成,     

经皮经肝介入门静脉血栓溶栓治疗

目的评价经皮经肝门静脉穿刺置管介入溶栓术的安全性和疗效。方法3例患者中,男性2例,女性1例,年龄分别为56岁,62岁,70岁。肝炎肝硬化2例,1例腹痛原因待查(后经手术证实为肠间脓肿)。3例患者均经彩色多普勒和CT检查发现门静脉血栓。3例患者均在B超引导下进行经皮经肝门静脉穿刺,置入5F CobraⅠ型导丝及导管鞘,经导管输注20万单位尿激酶后,继以每日30万单位持续注入,连续3天~5天行溶栓治疗,术后抗凝治疗。结果3例患者经皮经肝门静脉穿刺均一次成功,在置管造影及溶栓过程中均无不良反应;拔管前复查造影显示2例患者门静脉100%再通,1例80%再通;3例患者治疗后临床症状好转,腹水消退,肝功能改善,其中2例肝硬化患者食管静脉曲张程度减轻。1例腹痛原因待查患者溶栓后腹痛减轻但未完全缓解,体温较前下降但未正常,于溶栓后2周行剖腹探查,术中证实右下腹肠间包裹性脓肿,行脓肿切开引流,脓液培养为大肠杆菌,继续抗炎治疗2周后,体温降至正常,腹痛完全缓解。结论经皮经肝门静脉穿刺置管介入溶栓术是治疗门静脉血栓的安全、有效方法。     

Portal vein thrombosis with protein C-S deficiency in a noncirrhotic patient

There are several conditions that can lead to portal vein thrombosis(PVT), including including infection, malignancies, and coagulation disorders. Anew condition of interest is protein C and S deficiencies, associated with hypercoagulation and recurrent venous thromboembolism. We report the case of a non-cirrhotic 63-year-old male diagnosed with acute superior mesenteric vein thrombosis and PVT and combined deficiencies in proteins C and S, recanalized by short-term low molecular heparin plus oral warfarin therapy.     

Extrahepatic portal vein thrombosis: aetiology and determinants of survival

BACKGROUND: Malignancy, hypercoagulability, and conditions leading to decreased portal flow have been reported to contribute to the aetiology of extrahepatic portal vein thrombosis (EPVT). Mortality of patients with EPVT may be associated with these concurrent medical conditions or with manifestations of portal hypertension, such as variceal haemorrhage. PATIENTS AND METHODS: To determine which variables have prognostic significance with respect to survival, we performed a retrospective study of 172 adult EPVT patients who were followed over the period 1984-1997 in eight university hospitals. RESULTS: Mean follow up was 3.9 years (range 0.1-13.1). Overall survival was 70% (95% confidence interval (CI) 62-76%) at one year, 61% (95% CI, 52-67%) at five years, and 54% (95% CI, 45-62%) at 10 years. The one, five, and 10 year survival rates in the absence of cancer, cirrhosis, and mesenteric vein thrombosis were 95% (95% CI 87-98%), 89% (95% CI 78-94%), and 81% (95% CI 67-89%), respectively (n=83). Variables at diagnosis associated with reduced survival according to multivariate analysis were advanced age, malignancy, cirrhosis, mesenteric vein thrombosis, absence of abdominal inflammation, and serum levels of aminotransferase and albumin. The presence of variceal haemorrhage and myeloproliferative disorders did not influence survival. Only four patients died due to variceal haemorrhage and one due to complications of a portosystemic shunt procedure. CONCLUSION: We conclude that mortality among patients with EPVT is related primarily to concurrent disorders leading to EPVT and not to complications of portal hypertension.     

Liver transplant recipients with portal vein thrombosis:a single center retrospective study

BACKGROUND:Portal vein thrombosis(PVT)used to be a contraindication for liver transplantation(LT).This obstacle has been delt with following the improvement of LT-related techniques and therapeutic approaches to thrombosis. But the effect of PVT on LT outcomes is still controversial. We reviewed retrospectively the outcome of LT patients with PVT as well as risk factors and surgical management according to PVT grades. METHODS:A total of 465 adult LTs were performed from December 2002 through December 2006.O...     

Identification of two portal vein tumor thrombosis associated proteins in hepatocellular carcinoma: protein disulfide-isomerase A6 and apolipoprotein A-I

Background and Aim: Portal vein tumor thrombus (PVTT) is one of the factors that can affect prognosis and survival of hepatocellular carcinoma (HCC). In the present study, we aimed to find out some biomarkers associated with vascular invasion features of HCC with the method of comparative proteomic analysis. Methods: The proteins were extracted from a pair of HCC tissues with PVTT and without PVTT, and then separated by two‐dimensional polyacrylamide gel electrophoresis. Differentially expressed protein spots were identified by matrix assisted laser desorption ionization time‐of‐flight mass spectrometry (MALDI‐TOF MS). Further analysis of two proteins were completed using real‐time fluorescence quantitative polymerase chain reaction and western‐blot in 40 HCC tissues with PVTT (n = 20) and without PVTT (n = 20). Results: Among 465 protein spots displayed on the gels, 33 unique proteins (> twofold change, P < 0.01) were identified, including 24 upregulated in HCC tissue without PVTT and nine upregulated in HCC tissue with PVTT. The real‐time fluorescence quantitative PCR showed no statistically significant difference between HCC tissues with PVTT and without PVTT for mRNA expressions of protein disulfide‐isomerase, A6 (PDI A6) (P = 0.137) and apolipoprotein A‐I (Apo A‐I) (P = 0.718). However, compared with HCC tissues without PVTT, protein expression of PDI A6 was higher in HCC tissues with PVTT (P < 0.001), while protein expression of Apo A‐I was lower in HCC tissues with PVTT (P = 0.012). Conclusions: PDI A6 and Apo A‐I are closely related to vascular invasion feature of HCC.     

A case of portal vein thrombosis associated with acute cholecystitis/pancreatitis or coincidence

BACKGROUND: Portal vein thrombosis (PVT) is complex and risk factors include local precipitating factors and acquired and inherited factors. It occurs secondary to abdominal malignancy, infection or surgical intervention. PVT is commonly forgotten as a possible cause of abdominal pain. The clinical picture may vary but abdominal pain and low grade fever are the most characteristic picture. METHODS: A 58-year-old male patient was admitted to our hospital complaining of abdominal pain for three days. CT scan revealed an edematous area around the portal vein. Doppler ultrasonography showed evidence of a portal vein thrombosis. RESULTS: PVT can be diagnosed with CT and Doppler ultrasonography. Fresh thrombus can be undetected by sonography because of the low echogenity but can be recognised by color Doppler ultrasonography. Treatment ranges from observation and bowel rest to surgical resection of bowel. CONCLUSIONS: When we suspect a case of PVT, it should be treated at an early stage to prevent being lost in a diagnostic dilemma. The immediate use of anticoagulant could be important in preventing serious consequences of PVT.     

Cerebral sinus thrombosis in a patient with hereditary protein S deficiency: Case report and review of the literature

Summary Hereditary protein S deficiency is an established risk factor for venous thrombosis. The common sites of thrombosis are the deep leg and pelvic veins. We report on a 38-year-old female patient with hereditary protein S deficiency and a previous history of deep leg vein thrombosis, who developed thrombosis of the cerebral straight and superior sagittal sinus while taking oral contraceptives. The diagnosis was established by computerized tomography and carotid angiography. Lysis of the thrombus occurred during heparin treatment. The hereditary nature of protein S deficiency was documented by family studies, since nine additional family members deficient in protein S were identified. Nineteen published cases of cerebral vein thrombosis and a deficiency of either antithrombin III, protein C, or protein S were reviewed. Compared with patients without a deficiency state, the clinical features of cerebral vein thrombosis were similar except for an earlier onset and a positive medical history of venous thromboembolic events in a considerable number of patients.     

Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan.

BACKGROUND/AIMS: The aim of this study was to elucidate the incidence and clinical manifestations of portal vein thrombosis (PVT) in patients with idiopathic portal hypertension (IPH) in Japan during long-term follow-up. PATIENTS AND METHODS: Twenty-two patients with IPH were examined for PVT by sonography during a follow-up of 12+/-6 years. Clinical manifestations and patient outcome related to PVT were studied. Seventy patients with liver cirrhosis were examined by sonography as an incidence control of thrombosis. RESULTS: Nine IPH patients had portal thrombosis (9/22, 41%), a higher incidence than in liver cirrhosis patients (7/70, 10%). Those with thrombosis showed ascites, marked hypersplenism, and low serum albumin. Four patients with thrombosis died. Patients without thrombosis showed less clinical problems after long-term follow-up. Plasma antithrombin III and protein C activity decreased in almost half of the patients. However, there were no differences in these parameters between patients with and without thrombosis. CONCLUSIONS: In Japan, IPH patients had a high incidence of portal thrombosis, a significant factor for poor prognosis. Whether the management of PVT contributes to an improvement of a clinical course of IPH or not should be clarified in further study.     

Transient anticardiolipin antibodies, functional protein S deficiency, and deep vein thrombosis.

A 30-year-old man presented with an episode of deep vein thrombosis. He was found to have primary antiphospholipid syndrome with anticardiolipin antibodies and protein S deficiency. All other investigations were negative. Three months later, anticardiolipin antibodies were negative and protein S levels were normal. The transient presence of anticardiolipin antibodies and functional protein S deficiency in this patient suggests a new mechanism for the association between anticardiolipin antibodies and venous thrombosis.     

Chylous ascites caused by portal vein thrombosis treated with octreotide

Chylous ascites is an uncommon entity with variable causes and rarely arises from portal vein thrombosis. This is a case report of chylous ascites caused by idiopathic portal vein thrombosis that was refractory to medical therapy and shunt surgery, which showed an impressive response to treatment with subcutaneous octreotide. We review the literature on chylous ascites with particular reference to the role of somatostatin analogs in the management of this rare condition.     

Mesenteric artery thrombosis: A case report of combined protein S and protein C deficiency

Individuals with more than one defect in natural coagulant/anticoagulant systems have been postulated to be at an increased risk for thrombotic events. We report a case of combined protein S and C deficiency in a young woman, which resulted in fatal arterial mesenteric thrombosis. The role of coagulation defects in arterial thrombosis is discussed. Am. J. Hematol. 58:246–247, 1998. © 1998 Wiley-Liss, Inc.