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Hepatic vein thrombus is a common and often fatal complication of paroxysmal nocturnal haemoglobinuria. Rapid diagnosis with ultrasound, computed tomography or venography is required to improve the prognosis. Although heparin is often the initial therapy of choice, thrombolytic agents may be more beneficial in the early management.
Hepatic veno-occlusive disease (VOD) is a common complication of high-dose chemotherapy associated with bone marrow transplantation. While the pathogenesis of VOD is uncertain, plasminogen activator inhibitor-1 (PAI-1) has emerged as a diagnostic marker and predictor of VOD in humans. In this study, we investigated the role of PAI-1 in a murine model of VOD produced by long-term nitric oxide synthase inhibition using L-NAME. After 6 weeks, wild-type (WT) mice developed extensive fibrinoid hepatic venous thrombi and biochemical evidence of hepatic injury and dysfunction. In contrast, PAI-1-deficient mice were largely protected from the development of hepatic vein thrombosis. Furthermore, WT mice that received tiplaxtinin, an antagonist of PAI-1, were effectively protected from L-NAME-induced thrombosis. Taken together, these data indicate that NO and PAI-1 play pivotal and antagonistic roles in hepatic vein thrombosis and that PAI-1 is a potential target in the prevention and treatment of VOD in humans.
Hepatic venous outflow obstruction caused by hepatic vein thrombosis (HVT) is a manifestation of a hypercoagulable state, most commonly a myeloproliferative disorder (MPD). In the past, HVT was thought to have a poor prognosis unless treated surgically with portosystemic shunt or orthotopic liver transplantation (OLT). The aim of this study was to assess whether early diagnosis of the underlying hematologic disorder and institution of appropriate medical therapy have altered outcome. We reviewed the charts of 22 patients with HVT evaluated at our center from January 1986 to January 1995. The median age was 32 years (range, 14 to 59 years). Underlying etiologies were MPD, 13 (polycythemia vera, 8; essential thrombocythemia, 4; undefined, 1); dysfibrinogenemia, 1; anticardiolipin antibody, 1; oral contraceptive use, 3; and idiopathic, 4. All patients had ascites, hepatomegaly, and/or abdominal pain. Two underwent mesocaval shunting, and 1 had a peritoneal-venous shunt. Seven patients, including 1 with a mesocaval shunt, underwent OLT. The median duration of symptoms before transplantation was 6 months (range, 1.5 to 11 months). Six transplant patients are alive on long-term anticoagulation therapy at a mean post-OLT follow-up of 42 months (range, 2 to 77 months), without recurrence. Of 13 patients treated medically, 10 (77%) are alive at a median follow-up of 40 months (range, 17 months to 14 years 8 months), 1 has died, and 2 have been lost to follow-up. In a majority of patients, symptoms improve with prompt treatment of the underlying hematologic disorder, with a favorable long-term prognosis. Patients with decompensated liver disease can successfully undergo OLT with a low risk of recurrence on long-term oral anticoagulation.
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Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from obstruction to the outflow of blood from the liver. The characteristic pathologic findings are intense congestion most pronounced around the terminal hepatic venules, cell necrosis, and a scant inflammatory reaction. Major clinical manifestations include hepatomegaly, right upper quadrant abdominal pain, and ascites. Disorders associated with hepatic vein thrombosis include those associated with a thrombotic tendency, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria. Use of oral contraceptive agents probably increases tendency to develop hepatic vein thrombosis. Biochemical tests of the liver are of little value. The hepatic scan may be useful in suggesting the diagnosis with a marked decrease in uptake of isotope over affected areas of the liver. The diagnosis is confirmed by inferior vena caval and attempted hepatic venous catheterization. An associated thrombosis of the inferior vena cava is frequently found. Therapy in hepatic vein thrombosis is directed towards correction, in so far as possible, the underlying disorder, prevention of further extention of thrombosis, and management of ascites. Side-to-side portacaval or mesocaval shunt operation may convert the portal vein into an effective hepatic outflow vessel and reduce intrahepatic pressure and decrease injury. Mesoatrial shunts have proven useful in a few patients with hepatic vein thrombosis and either an inferior vena caval thrombosis or a marked pressure gradient from the abdomen to the thorax from ascites and an hypertrophied caudate lobe of the liver. For patients with extensive hepatocellular disease and portal hypertension, hepatic transplantation would seem to offer the best chance for survival.
We present a patient with paroxysmal nocturnal hemoglobinuria with diffuse hepatic central vein thrombosis who presented with encephalopathy and recovered from the hepatic manifestations with antihepatic coma measures alone.
Hepatic vein thrombosis initiated by an intravenous injection of endotoxin (Salmonella typhosa, 0.3 mg/kg) resulted in an incidence of 78% in rats fed a butter-rich diet (group 1). On the other hand, no lesion could be produced in control animals fed corn oil (group 2) or standard chow (group 3). In regard to the respective thrombotic tendencies, the rats fed butter showed higher circulating levels of factor XII (175% vs 140% in group 2 and 100% in group 3) and a far more severe decrease in this factor (41% vs 15% in group 2 and 7% in group 3) and in platelets (48% vs 25% in group 2 and 19% in group 3) 2 hours after the injection of endotoxin. The triggering effect of endotoxin could be reproduced by ellagic acid, a known activator of factor XII. Given by slow infusion (1 mg/kg/min) this chemical induced hepatic vein thrombosis in 52% of the rats fed the butter-rich diet. Furthermore, inhibition of factor XII activation by lysozyme (20 mg/kg/min) completely prevented hepatic vein thrombosis initiated by endotoxin in butter-fed animals. It is concluded that, in addition to the potent hypercoagulability induced by the fat-rich diets, activation of Hageman factor consecutive to endotoxin injection is essential for production of the phenomenon of hepatic vein thrombosis.
BACKGROUND/AIMS: Hepatic vein thrombosis is thought to be manifested by ascites, abdominal pain, and hepatomegaly, with a uniformly poor prognosis. However, new imaging techniques allow for the diagnosis of hepatic vein thrombosis in asymptomatic cases. The aim of our study was to re-evaluate symptoms and prognosis in patients with hepatic vein thrombosis. METHODS: Eighty-one patients with hepatic vein thrombosis were analyzed. Forty-seven patients were admitted from 1970 to June 1987 (group I, before Doppler ultrasonography and magnetic resonance imaging were introduced at our hospital) and 34 from July 1987 to June 1991 (group II). RESULTS: When comparing the two groups, age, sex ratio, and causes of hepatic vein thrombosis did not differ. Eight group II patients (asymptomatic patients) had no ascites, hepatomegaly, or abdominal pain. One major hepatic vein remained patent in 41% of group II patients, compared with 12% in group I (P < 0.05). Intrahepatic collaterals were seen in 79% of group II patients, compared with 21% of group I patients (P < 0.01). All asymptomatic patients had large intrahepatic and portasystemic collaterals. At 3 years, death occurred in 22% of group II patients and in 45% of group I patients. No asymptomatic patient died. CONCLUSIONS: Asymptomatic hepatic vein thrombosis is associated with the spontaneous development of large intrahepatic and portosystemic collaterals. In asymptomatic patients, prognosis at 3 years seems to be good, and surgical therapy may not be required.
We describe four patients with hepatic vein thrombosis caused by Behçet's disease and review the 17 previously published cases. In addition, we compared these 21 cases of hepatic vein thrombosis to our 24 cases of hepatic vein thrombosis caused by primary myeloproliferative disorders. In patients with Behçet's disease, a male predominance (male/female ratio, 19:1) contrasted with the female predominance found in patients with hepatic vein thrombosis complicating primary myeloproliferative disorders (sex ratio = 1:3). The mean age at clinical onset was younger in patients with Behçet's disease than in those with primary myeloproliferative disorders (29 vs. 35 yr). Obstruction of the inferior vena cava was found in 90% of patients with hepatic vein thrombosis caused by Behçet's disease. Inferior vena caval thrombosis appears to be the main pathophysiological mechanism of hepatic vein thrombosis in patients with Behçet's disease. Unlike patients with primary myeloproliferative disorders who often had a progressive course, one third of patients with Behçet's disease had acute liver failure and died within 2 wk of clinical onset. These findings suggest that, in patients with Behçet's disease, hepatic vein thrombosis is a sudden event usually related to the extension of a caval thrombus to the ostium of the hepatic veins.
Budd-Chiari syndrome is a rare disease due to occlusion of the hepatic veins often presenting with acute liver failure. Common causes of Budd-Chiari syndrome are chronic myeloproliferative disorders, while acute leukemia has been associated with hepatic vein thrombosis in only two cases in the literature to date. We report a case of Budd-Chiari syndrome complicating a non-promyelocytic acute myelogenous leukemia leading to fulminant hepatic failure.
Hepatic vein thrombosis, as well as hepatocellular carcinomas, was induced in inbred C3H male and female mice ingesting 10 ppm of dieldrin, aldrin, heptachlor, or heptachlor epoxide in the diet. Thrombosis was present in 5% of mice ingesting dieldrin or aldrin and in 10.5% of mice ingesting heptachlor or heptachlor epoxide. Occlusion of the hepatic vein often resulted in infarcts of the liver. Females ingesting heptachlor or heptachlor epoxide were slightly more susceptible than males. There was no difference between male and female mice ingesting dieldrin or aldrin. Hepatic vein thrombosis did not appear to be related to the development of carcinoma of the liver because it was present in livers without carcinomas as well. Thrombosis was usually seen only in the liver but also rarely was present in the atria of the heart.
Hepatic vein thrombosis is caused by one or several thrombogenic conditions, of which myeloproliferative disorders are the most frequent. Thrombosis and its fibrous sequelae can affect the veins diffusely or locally. Severity is determined by the extent and velocity of the thrombotic process. Development of venous collateral vessels is an important compensatory mechanism. Some patients can be totally free of symptoms. Major complications are intractable ascites, liver insufficiency, and gastrointestinal bleeding. Diagnosis can be made via ultrasonography or magnetic resonance imaging in a majority of cases. The main prognostic factors are age, Child-Pugh score, and response of ascites to diuretics. Medical therapy includes control of causal factors, anticoagulation, and nonspecific treatment of complications. Procedures aiming to restore outflow of hepatic blood are indicated in patients with uncontrolled manifestations. Percutaneous angioplasty, followed by portosystemic shunt (including a transjugular intrahepatic portosystemic stent shunt) and eventually liver transplantation can be proposed in a graded manner. The current 10-year survival rate is about 75%.
Hepatic vein thrombosis or the Budd-Chiari syndrome is an uncommon condition which rarely occurs in teenagers. The condition involves obstruction of blood flow from the liver with progressive congestion and hepatic failure. The etiology is often idiopathic but may involve conditions that increase thrombogenic tendencies. Oral contraceptives are known to increase the risk of a number of thrombotic conditions and have been associated with the Budd-Chiari syndrome. Although millions of teenagers have used oral contraceptives without incident, occasionally we are reminded of their potential serious complications. The case of a 19-year-old who developed hepatic vein thrombosis while using oral contraceptives is presented.
It has been suggested in previous studies, that inflammatory bowel disease can induce hepatic vein thrombosis. However, the main weakness of those studies was the lack of extensive screening of prothrombotic factors. We report an unusual association of severe inflammatory bowel disease, hepatic vein thrombosis and latent platelet proliferation disorder. Early treatment with heparin and cyclosporin administered intravenously induce a remission of intestinal disease and total disappearance of hepatic vein thrombosis. Extensive screening excluded the classical causes of hepatic vein thrombosis, inherited coagulation disorders and latent polycythemia by culture of erythroid progenitors without added erythropoietin. However, using recent bone marrow culture conditions, we observed spontaneous colony formation of megakaryocyte progenitors revealing latent thrombocythemia. In summary, progress in the diagnosis of haemostasis disorders, will probably confirm that one or more additional predisposing prothrombotic factors are needed to the development of hepatic vein thrombosis in inflammatory bowel disease. This hypothesis could explain the scarcity of this complication in inflammatory bowel disease. Moreover, this case-report suggests that administration of heparin at the onset of the thrombosis may induce a complete regression of the thrombus in hepatic veins.
Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins, but subsequent reports on various obliterative changes that occur in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices have resulted in a broadened and ambiguous definition. Membranous obstruction of the inferior vena cava has been regarded by many as a congenital vascular malformation, but its relation to the classical BCS has remained obscure. With modern imaging and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to classify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review, we emphasize that primary hepatic vein thrombosis (classical Budd-Chiari) and an obliterative disease predominantly affecting the hepatic portion of the IVC, both of which account for most patients with venous outflow block, are clinically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow block in developing countries such as Nepal, South Africa, China, and India. The latter is frequently complicated by hepatocellular carcinoma (HCC), and primary hepatic vein thrombosis is not. The major cause of thrombosis is a hypercoagulable state in hepatic vein thrombosis, but more of the latter cases are idiopathic. The clinical presentation of the latter is milder, and onset is frequently inapparent, whereas the former is more severe, sometimes causing acute hepatic failure. Markedly enlarged subcutaneous veins over the body trunk characterize the latter. We propose that these two disorders be clinically distinguished with a suggested term "obliterative hepato-cavopathy" for the latter against classical BCS.
Venous thrombophilia is the result of clotting changes namely of a hypercoagulable state together with blood flow and vessel wall changes. There is no need for all these components to be present in order for thrombosis to occur. As the matter of fact, thrombosis may occur even if only one of these conditions is present. In clinical practice a combination of factors is usualy seen. In comparison with arterial thrombophilia, clotting changes and blood flow seen to play a major role in venous thrombosis. Venous thrombophilia may remain asynptomatic or may result in a series of clinical syndromes. The commonest of these are: 1. Superficial vein thrombosis, 2. Deep vein thrombosis of legs, 3. Deep vein thrombosis of arms, 4. Caval veins thrombosis, 5. Portal vein thrombosis, 6. Hepatic veins thrombosis, 7. Renal vein thrombosis, 8. Cerebral sinuses thrombosis, 9. Right heart thrombosis, 10. Miscellaneous (ovarian, adrenal veins thrombosis, etc.). Since the first two are widely and easily recognized, these is no need for an extensive discussion. Deep vein thromboses of upper limbs are not as frequent as those of lower limbs or of superficial phlebitis but they can still be recognized on clinical grounds and non invasive techniques. The remaining 7 syndromes are less common and therefore less frequently suspected and recognized. Of particular interest, among these less common manifestations of venous thrombophilia are hepatic vein and renal vein thrombosis. Hepatic veins thrombosis, sometimes part of inferior vena cava thrombosis is most frequently due to an isolated occlusion of hepatic veins thereby causing a form of venocclusive disease. Occasionally diagnosis may be difficult because of slow onset of symptoms (hepatomegaly, right flank pain, fever, ascites etc.). The same is true for renal vein thrombosis which may also be of difficult diagnosis since it causes proteinuria and flank pain. The proteinuria is often interpreted as due to a nephrotic syndrome which, incidentally, may cause by its turn renal vein thrombosis. Portal vein thrombosis and cerebral sinuses thrombosis on the contrary are more easily suspected because of ascites, adominal pain, jaundice or headache, eye proptosis, vomiting. Right heart thrombosis should be suspected in cases of recurrent pulmonary embolization. Ovarian or adrenal veins thrombosis are rare. The competent physician should always consider, given certain congenital or acquired conditions, the possibility to be facing a special form of venous thrombosis or a venous thrombosis in unusual sites. An early diagnosis, as often in medicine, is of paramount importance for a prompt treatment and a satisfactory outcome.
A 22-year-old female with active ulcerative colitis developed massive ascites, hypoalbuminemia, and hepatomegaly compatible with thrombosis of the hepatic veins. The diagnosis of Budd-Chiari syndrome was confirmed by ultrasonography, computed tomography, and by liver biopsy. A search of the literature disclosed only three previous reports of Budd-Chiari syndrome occurring in patients with ulcerative colitis. All patients have been young females with active colitis and no other known risk factor for the development of hepatic vein thrombosis. Our patient, unlike the previously reported patients who died, recovered sufficiently to be discharged from the hospital.
In a case-control study, we evaluated the relative risk of hepatic vein thrombosis among recent oral contraceptive users as compared with nonusers. Thirty-three cases of hepatic vein thrombosis affecting women aged 15-45 yr were collected between 1970 and 1983, and individually matched to 3 or 4 controls interviewed in 1982-1984. There were 18 recent oral contraceptive users among the 33 cases, and 44 recent oral contraceptive users among the 128 case-matched controls. The relative risk of hepatic vein thrombosis was 2.37 (95% confidence interval: 1.05-5.34, p less than 0.02). The relative risk of hepatic vein thrombosis is close to that of stroke, myocardial infarction, and venous thromboembolism among oral contraceptive users as compared with nonusers.