Portal and mesenteric vein and inferior vena cava thrombosis associated with antiphospholipid syndrome

We report a 48-year-old man with thrombosis of the portal and superior mesenteric vein and inferior vena cava associated with primary antiphospholipid syndrome (APS). Primary APS was diagnosed by a positive reaction with anticardiolipin antibody (aCL) and the absence of any evidence suggesting the presence of other disease states known to be associated with aCL. A coeliac angiography showed obstruction of the portal and superior mesenteric vein with prominent collaterals and cavernous transformation. Femoral vein angiography showed total obstruction of the external iliac vein and inferior vena cava, and dilation of the pelvic veins, with contrast medium in the lumbar vein. This case is noteworthy as a report of primary APS accompanied by extensive abdominal and pelvic venous thrombosis.     

Risk of thrombosis in patients with antiphospholipid antibodies and factor V Leiden mutation

OBJECTIVE: Antiphospholipid antibodies (aPL) are thrombophilic risk markers in patients with systemic lupus erythematosus (SLE) or primary antiphospholipid syndrome (APS). The risk factors for recurrent venous or arterial thrombosis and indications for longterm anticoagulation therapy are debated. We hypothesized that carrying a second thrombophilic defect, factor V Leiden mutation, would increase the risk for thrombosis in patients with aPL. METHODS: Seventy-five patients with primary APS and 83 with SLE and aPL with or without thrombosis followed at 2 university hospitals were studied. Factor V mutation rate was analyzed in patients and in 200 healthy blood donors by polymerase chain reaction analysis. RESULTS: The prevalence of factor V Leiden mutation in patients with SLE and aPL or primary APS was similar to controls. Patients with deep vein thrombosis or arterial thrombosis did not have a significantly increased rate of factor V mutation compared to controls or to patients with aPL without thrombosis. CONCLUSION: Factor V Leiden mutation is not significantly associated with vein thrombosis in patients with aPL. However, due to the sample size we cannot rule out synergy between both factor V Leiden and aPL. A trend toward increased risk for thrombosis was detected in patients with the mutation and this should be analyzed in a larger study.     

Acute thrombosis after elective direct intracoronary stenting in primary antiphospholipid syndrome: a case report

Antiphospholipid syndrome (APS) is an uncommon prothrombotic disorder that has been increasingly recognized in recent years. The diagnosis of APS must be associated with venous or arterial thrombosis or both. Patients with APS usually present with recurrent deep vein thrombosis, pulmonary thromboembolism, thromboembolic stroke, or myocardial infarction. Here, we report a case of a 61-year-old female who presented with a 3-month history of increasingly frequent retrosternal chest tightness. After treadmill test and thallium-201 myocardial perfusion scan, she was admitted and underwent elective coronary angiography but developed acute thrombosis after direct intracoronary stenting. She was successfully rescued with repeat percutaneous transluminal coronary angioplasty and prolonged heparin and glycoprotein IIb/IIIa antagonist use. Laboratory data showed prolongation of partial thromboplastin time and positive anti-cardiolipin antibody. These findings satisfied the criteria for APS; the patient was diagnosed with primary APS because she had neither typical symptoms nor signs of systemic lupus erythematosus or other immunologic disorders. Thereafter, long-term oral anticoagulant appeared to be effective. To our knowledge, this is the first report of acute stent thrombosis in a patient with primary APS.     

Primary antiphospholipid syndrome: a cause of fever of unknown origin

Antiphospholipid syndrome (APS) is defined as the occurrence of thrombosis, recurrent miscarriage, or both in association with laboratory evidence of persistent antiphospholipid antibodies. Owing to protean manifestations and laboratory studies, the diagnosis may be difficult. Because the other signs and symptoms of thrombosis are predominant, prolonged fever is not usually the main clinical finding. We describe a patient who presented with fever of unknown origin (FUO) and was found to have thromboses of the splenic vein, the superior mesenteric vein, and the portal vein due to the primary antiphospholipid syndrome. We also reviewed the medical literature (Medline 1966-2001), including the main FUO series of the previous 40 years, and laparotomy series for FUO. We conclude that although very rare, primary APS and thrombosis may present with FUO. APS should be considered in the differential diagnosis of prolonged fever associated with thrombosis.     

Clopidogrel and aspirin after ischemic stroke or transient ischemic attack: an updated systematic review and meta-analysis of randomized clinical trials

Journal of Thrombosis and Thrombolysis - Patients with primary or secondary antiphospholipid syndrome (APS) have an increased risk of recurrent venous, arterial thrombosis and pregnancy...     

Antiphospholipid syndrome

Antiphospholipid syndrome (APS) is characterized by recurrent arterial or venous thromboembolism or pregnancy loss in association with antibodies directed against anionic phospholipids or plasma proteins bound to anionic phospholipids. A common cause of the huge variety of clinical manifestations is vaso-occlusive disease and not vasculitis in venous or arterial blood vessels of different sizes and sites (i.e. deep vein thrombosis, pulmonary embolism, cerebrovascular disease). In accordance with this, fetal abortion, typically beyond the tenth week of gestation, is also caused by infarctions of blood vessels in the placenta. Establishing the correct diagnosis of APS is not easy. To estimate the risk of thrombotic complications is challenging, as well as the questions of, which, how long and in what strength anticoagulation is recommended. This paper should enable the reader to apply international consensus classification criteria correctly, to interpret the different laboratory tests for anti-phospholipid antibodies and to gain an awareness of the different forms of anticoagulation in order to stratify therapeutic decisions.     

Antiphospholipidsyndrom

Antiphospholipid syndrome (APS) is characterized by recurrent arterial or venous thromboembolism or pregnancy loss in association with antibodies directed against anionic phospholipids or plasma proteins bound to anionic phospholipids. A common cause of the huge variety of clinical manifestations is vaso-occlusive disease and not vasculitis in venous or arterial blood vessels of different sizes and sites (i.e. deep vein thrombosis, pulmonary embolism, cerebrovascular disease). In accordance with this, fetal abortion, typically beyond the tenth week of gestation, is also caused by infarctions of blood vessels in the placenta. Establishing the correct diagnosis of APS is not easy. To estimate the risk of thrombotic complications is challenging, as well as the questions of, which, how long and in what strength anticoagulation is recommended. This paper should enable the reader to apply international consensus classification criteria correctly, to interpret the different laboratory tests for anti-phospholipid antibodies and to gain an awareness of the different forms of anticoagulation in order to stratify therapeutic decisions.     

Antiphospholipid syndrome: state of the art with emphasis on laboratory evaluation

Antiphospholipid antibodies (aPLs) are associated with arterial and venous thrombosis, recurrent pregnancy loss and thrombocytopenia. Although aPLs have not yet been conclusively shown to be causal in thrombosis and miscarriage, they are useful laboratory markers for the antiphospholipid syndrome (APS). The syndrome can complicate another autoimmune disease, most commonly systemic lupus erythematosus, but more often occurs alone -- primary APS. Identification of the syndrome is clinically important because of the risk of recurrent thrombosis and the need for antithrombotic therapy in many cases. Diagnosis and treatment of APS represent significant challenges, however, owing to the protean clinical manifestations and associations, limitations of currently available laboratory tests for aPLs, and the lack of clear evidence-based guidance on optimal management.     

A unique presentation of antiphospholipid antibody syndrome in pregnancy

Primary antiphospholipid antibody syndrome (APS) is a protean disease with many manifestations including venous and arterial thrombosis, recurrent foetal loss, preeclampsia, intrauterine growth retardation, cardiac valvular disease, glomerulonephritis, thrombocytopaenia and livedo reticularis. We report an interesting case of a 19-year-old woman where the diagnosis of primary APS was initially made in the peripartum period.     

Portal venous occlusion

Summary Morbidly obese patients are significantly more susceptible to clotting phenomena, including recurrent deep venous thrombosis, pulmonary emboli, inferior vena caval thrombosis, and renal vein thrombosis. The patient described in this report developed the sudden onset of massive ascites two years following jejunoileal bypass for morbid obesity. Occlusion of the portal vein and its tributaries was demonstrated. Our experience and one previous report suggest that portal vein thrombosis may be a rare complication of this surgical procedure.     

Catastrophic antiphospholipid syndrome associated with malignancies (case report and review of the literature)

We describe a 58-year old female patient with rapid development of arterial and venous thromboembolisms, including deep vein thrombosis (DVT) in the lower limbs, recurrent cerebral infarctions and bilateral pulmonary emboli. Her laboratory data on admission showed positive anticardiolipin antibody of IgG isotype (IgG aCL) and positive anti-beta2 glycoprotein-I antibody of IgG isotype (IgG abeta2-GPI), and decreased protein C activity and protein S antigen. Systemic examinations revealed the presence of an ovarian cancer. Surgical resection was attempted, but her cancer infiltrated the pelvic wall and could not be resected. Despite treatment with unfractionated heparin followed by warfarin, she died due to recurrent episodes of cerebral infarction. This case was considered as probable catastrophic antiphospholipid syndrome (CAPS), which might be associated with ovarian cancer. Known as Trousseau's syndrome, arterial and, more commonly, venous thrombosis is a frequent complication of cancer and sometimes a harbinger of occult cancer. Our case indicates that there is an overlap between antiphospholipid syndrome (APS) and Trousseau's syndrome. It is important to bear in mind that a thrombotic event associated with cancer can be the first manifestation of CAPS.     

Cavernous Transformation of the Portal Vein: A Common Manifestation of Behcet's Disease

Behcet's disease (BD) is a chronic, multisystem inflammatory disorder of unknown etiology, which is characterized by recurrent aphthous ulcers of the mouth and genitalia, uveitis with hypopyon, and a diffuse vasculitis that involves the arterial and venous systems.
From January 1968 to July 1993, 66 of 844 patients with BD seen at the Hacettepe University Hospital, Ankara, Turkey, experienced a vascular complication other than peripheral thrombophlebitis. The vascular complication in each case was identified based upon a combination of clinical data, digital subtraction angiography, CT, and ultrasonography findings. Six of these 66 (9.1%) had cavernous transformation of the portal vein. Five of these six had additional large vein involvement resulting in the Budd-Chiari syndrome with or without inferior vena caval obstruction.
Based upon this experience, it can be concluded that portal vein thrombosis is not a rare complication of BD. When patients with BD are found to have or develop splenomegaly, portal vein thrombosis should be suspected and investigated. If hepatomegaly and ascites are detected, Budd-Chiari syndrome due to hepatic vein thrombosis should be suspected. Finally, if hepato-splenomegaly, ascites, and dependent edema of the lower body are present, thrombosis of the inferior vena cava should be suspected.     

Cholestasis as presenting symptom of portal cavernoma

A 51-year-old man with a history of portal vein thrombosis, was examined because of elevated liver tests and a tumoral mass in the liver hilus. Computed tomography (CT) scan and magnetic resonance imaging confirmed the portal vein thrombosis and an infiltrating mass in the porta hepatis with compression on the common bile duct. Endoscopic retrograde cholangiography showed an irregular narrowing of the mid-part of the common bile duct. The patient was referred for explorative laparotomy, which revealed a hypervascular mass in the liver hilus surrounded by many blood vessels. The diagnosis of portal cavernoma was made. Further haematological examination for the cause of portal vein thrombosis revealed an anti-phospholipid syndrome as well as myeloproliferative disease. Oral anticoagulant treatment is started. In conclusion, we report a case of biliary stricture due to portal vein thrombosis and cavernoma (portal biliopathy) which was not diagnosed preoperatively. Biliary strictures associated with portal vein thrombosis are due to extrinsic compression by collaterals and can also be induced by ischemic injury secondary to venous and arterial thrombosis of the choledochal vascular plexus.     

Clusters of disease manifestations in patients with antiphospholipid syndrome demonstrated by factor analysis

The antiphospholipid syndrome (APS) is now recognized as a multi-system disease, the clinical expression of which may include various target-organs involvements. Despite the reported heterogeneity in clinical presentation of APS, the interrelations between various manifestations of the disease has not yet been studied. We evaluated the principle associations between a variety of clinical manifestations in APS patients, applying factor analysis. Two-hundred and forty-six APS patients were studied. The following disease manifestations were used for the factor analysis: recurrent fetal loss, intrauterine growth restriction (IUGR), venous and arterial thrombosis, cardiac valves thickening/dysfunction, valvular vegetations, stroke, epilepsy, migraine, arthritis, livedo reticularis, thrombocytopenia, leukopenia and autoimmune hemolytic anemia (AIHA). The results were further analysed in relation to sex and to primary APS versus APS associated with SLE. Five factors were derived, which accounted for 59.7% of the variance of the matrix. Factor 1 (which explained 18.5% of variance of the original matrix) represented the association between cardiac valves abnormalities, livedo reticularis and AIHA. Factor 2 (13.8% of variance) represented association between arthritis, thrombocytopenia and leukopenia. Factor 3 (10.3% of variance) represented an association between recurrent fetal loss and IUGR. Factor 4 (9.3% of variance) represented inverse correlation between arterial and venous thrombosis. Factor 5 (7.8% of variance) represented an association between epilepsy and migraine. Application of factor analysis revealed specific clusters of cardiac, cutaneous, hematological and neurological manifestations. Our result also point to a possible divergence of arterial and venous thrombotic tendency. Awareness of these patterns might give us a better understanding of the disease.     

Antiphospholipid syndrome

Antiphospholipid syndrome (APS) is an autoimmune condition characterized by the occurrence of thrombosis (arterial and/or venous), often multiple, and/or pregnancy morbidity.Thrombosis is one of the major disease mechanisms, mainly caused by activating endothelial cells, monocytes, and platelets. At present, the management of APS patients with a history of thrombosis is based on long-term antithrombotic therapy, due to the high rate of recurrent thrombosis (29% per year without treatment). Obstetrical APS includes heterogeneous pregnancy complications whose pathogenesis has been increasingly elucidated in the past years. This is due to the current management and treatment, as 80% of APS patients achieve a live birth. The standard approach of APS is not supported by extensive evidence and the best options for refractory and incomplete cases need to be clarified. New and promising molecules are under investigation.     

Antiphospholipid syndrome: an evolving story

Antiphospholipid Syndrome (APS) is a form of immune mediated thrombophilia, presenting as recurrent thrombotic events and pregnancy morbidity, in association with positive laboratory tests for antiphospholipid antibodies (APA) in the form of Lupus Anticoagulant (LA) or anticardiolipin antibodies(ACA). Insights into the pathophysiology of the condition suggest that some antibodies are prothrombotic in vivo, and that the mechanism of thrombosis is likely to be multifactorial. APS has a broad spectrum of clinical presentations, and the laboratory diagnosis can be difficult due to heterogeneity of APAs and poor standardisation of laboratory tests. Anticoagulation is the mainstay of the management of the thrombotic and obstetric complications of APS. The risk of recurrent thrombosis appears to be high, and the duration and intensity of therapy remains controversial. Randomised controlled trials have shown that standard intensity anticoagulation is adequate in most cases of venous thrombosis. Further trials are required to establish whether high intensity coagulation is of benefit in recurrent or arterial thrombosis. The optimal management of recurrent fetal loss is debated and large studies are required to establish a clear benefit of heparin and aspirin over aspirin alone or supportive care.     

Stroke and antiphospholipid syndrome: the treatment debate

Antiphospholipid syndrome (APS) is one of the most frequent acquired thrombophilias. Thromboses can be at both the venous and arterial level, are usually recurrent and frequently affect cerebral circulation. Although it is difficult to predict which patients with antiphospholipid antibodies (aPL) will develop thrombosis, once a thrombotic event has taken place, secondary prevention is mandatory. Recommendations for treatment of APS have long been based on studies with a retrospective design. Recently, three prospective studies (two controlled and one uncontrolled small series) have addressed the role of antiaggregant and anticoagulant therapy in patients with stroke and aPL. However, results from prospective and retrospective studies are not in full agreement. In addition to the obvious differences in design, other factors such as the important variability in the study groups account for the discrepancy. While future investigation must better define homogeneous subsets of patients with APS, current secondary prophylaxis of thrombosis in these patients must be tailored according to individual estimated risk of recurrences, risk of haemorrhage and severity of potential recurrent events. Due to the often devastating consequences of stroke, we believe there is a place for prolonged, high-intensity anticoagulation in patients with APS and cerebral arterial thrombosis.     

Hepatic arterial blood flow in large hepatocellular carcinoma with or without portal vein thrombosis: assessment by transcutaneous duplex Doppler sonography

BACKGROUND: As liver cirrhosis progresses, the portal venous blood (PVBF) flow decreases, accompanied by an increase in hepatic arterial blood flow. Large hepatocellular carcinoma is a hypervascular tumour with a rapid growth, which seems to require an increase of the tumoral arterial blood flow. Furthermore, hepatocellular carcinoma is frequently associated with portal vein thrombosis, which subsequently impedes portal blood supply. METHODS: The purpose of our study was to estimate alterations in the hepatic arterial blood flow in large hepatocellular carcinomas occurring in liver cirrhosis, in comparison with liver cirrhosis and controls. Liver blood flow measurements were determined by duplex Doppler sonography in 47 patients with large hepatocellular carcinomas (13 with portal vein thrombosis and 34 without this thrombosis), 42 liver cirrhosis patients and 30 controls. The Doppler perfusion index was calculated as the ratio of hepatic arterial blood flow to total hepatic blood flow. RESULTS: The patients with liver cirrhosis had a significant increase of hepatic arterial blood flow as compared to controls (P < 0.001), accompanied by a significant reduction in PVBF (P < 0.005). As a result, the Doppler perfusion index was increased in patients with liver cirrhosis as compared to controls (P < 0.001). The hepatic arterial blood flow was increased in patients with hepatocellular carcinoma but without portal vein thrombosis as compared to the cirrhotic patients (P < 0.001), with a significant reduction of PVBF (P < 0.001). Hepatic arterial blood flow was also increased in patients with both hepatocellular carcinoma and portal vein thrombosis as compared to the patients without this thrombosis (P < 0.001). CONCLUSION: These results suggest that in large hepatocellular carcinomas there is a decreased PVBF, accompanied by an increased hepatic arterial blood flow. The hepatic arterial buffer response seems to be active in hepatocellular carcinomas and maintains liver perfusion to adequate levels.