Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Screening for red cell defects, and exploration of cellular and humoral immunity has been performed in 33 patients : 31 had agnogenic myelosclerosis with myeloid metaplasia, 3 had polycythemia vera with secondary myelosclerosis. No patient had the biological abnormalities characteristical of paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli). In 19 out of 21 cases, red cells had antigen i on their membrane, thus suggesting that splenic erythropoiesis could give rise to immature erythrocytes. Two patients had a monoclonal dysglobulinemia, 5 a positive Coombs test, 6 a rhumatoid factor in the serum, 3 antitissue antibodies, 1 LE cells, 3 a positive Paul-Bunnel-Davidsohn test without mononucleosis, 11 a negative skin test. Implications of the uncommon occurrence of these dissorders are discussed.
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PMID:[Erythrocytic and immunologic abnormalities in myeloid splenomegaly]. 60 Jul 81

Red cell membrane glycopeptides of subjects suffering from different hematological disorders (PNH, hemolytic anemias, dyserythropoietic anemias and polycythemia vera) have been characterized. In most cases, except in polycythemia vera, a decrease of sialic acid and galactosamine was detected. The role of these alterations is discussed with regard to the decrease of membrane glycopeptides during physiological aging of the red cell.
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PMID:Glycopeptides of erythrocyte membranes in some hematological disorders. 82 Dec 92

Twenty-six patients with the Budd-Chiari syndrome were treated surgically at the Johns Hopkins Hospital. Twenty-one of the patients were female and five were male, with a median age at diagnosis of 37 years. Nine patients had polycythemia vera, 6 were receiving estrogen therapy, 5 had a previous hepatitis A or B infection, and 4 had cirrhosis. There was one case each of hepatic malignancy, paroxysmal nocturnal hemoglobinuria, and idiopathic thrombocytopenic purpura. In five cases no etiologic factors or associated disorders were identified. Ascites was the most common presenting feature in this group of patients. Hepatic function at the time of diagnosis, as measured by standard serum chemistries, was only minimally abnormal. The diagnosis of the Budd-Chiari syndrome was confirmed in all 26 patients by hepatic vein catheterization. Inferior vena cavography was also performed and revealed caval occlusion in 4 patients, significant caval obstruction in 13 patients, and a normal vena cava in 9 patients. Interpretation of the vena cavogram was helpful in selecting the appropriate surgical procedure for each patient. Twenty-three of the twenty-six patients underwent percutaneous liver biopsy before operation, with no morbidity or mortality. Four patients had well-established cirrhosis noted on biopsy. Thirty mesenteric-systemic venous shunts were performed on the 26 patients. In 11 patients a mesocaval shunt was performed and in one instance conversion to a mesoatrial shunt was required as a second procedure. In 15 patients a mesoatrial shunt was performed as the initial procedure. Graft thrombosis occurring in 2 of these 15 patients prompted one revision in 1 patient and 2 revisions in the second patient. After mesenteric-systemic venous shunt, eight of the patients (31%) died before discharge from the hospital. The remaining 18 patients in this series were discharged from the hospital alive and well with patent shunts. Patients were followed for a median of 43 months (range, 9 months to 13 years). Five late deaths occurred between 5 and 84 months after the operation. Three- and five-year actuarial survival rates were 65% and 59%, respectively.
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PMID:Current management of the Budd-Chiari syndrome. 200 20

We have developed a RIA for erythrocyte acid glutathione S-transferase (GST), which is immunologically identical to major GSTs from other blood cell components, and measured its serum concentrations in various hematological disorders. In some patients with paroxysmal nocturnal hemoglobinuria, chronic myelomonocytic leukemia, chronic myelocytic leukemia, polycythemia vera and myelofibrosis, the concentrations were high. Very high levels were found in 2 of 3 patients with acute lymphocytic leukemia, while acute myelocytic leukemia exhibited a modest increment. No or little increase was seen in aplastic anemia and myelodysplastic syndrome except chronic myelomonocytic leukemia. It is suggested that the measurement of serum acidic GST may be of use as a clinical marker of increased destruction and/or overproduction of blood cells.
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PMID:Radioimmunoassay for erythrocyte acidic GSH S-transferase. 249 36

The Budd-Chiari syndrome is a rare condition (0.4-.06 per cent in autopsy material) characterized by ascites, liver function disturbance and abdominal pain caused by thrombosis of the major hepatic veins. $ studies (N = 114) yield the following list of causes with percentages; Oral contraceptives, 18%; polycythemia vera, 13%; other myelo-proliferative disease, 4%; paroxysmal nocturnal hemoglobinuria, 5%; blood vessel malformation, 10%; malignancy, 6%; other simultaneous thrombosis, 3%, vasculitis, 2%; other (trauma, abscess, chronic active hepatitis, pregnancy) 5%; no known cause, 34%. The histories of 2 patients illustrate the difficulty of diagnosis, which is usually verified only by biopsy. One of the patients was a 20-year old woman who had used oral contraceptives for 5 years and presented changes consistent with myeloproliferative syndrome in the peripheral circulation and in the bone marrow, as well as a high cardiolipin antibody titer. Oral contraceptives have been cited as a cause of Budd- Chiari syndrome, but the proportion of oral contraceptives users among patients is no greater than among women in general. One recent French study (N = 33) gives a relative risk factor of 2.4 for women between 15 and 45 years old who have used oral contraceptives during the 12 months before onset of the disease. This risk factor parallels that for stroke, myocardial infarction, and venous thromboembolism. No cases of Budd- Chiari syndrome had been reported to the Swedish side-effects register through December 1988.
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PMID:[Oral contraceptives and blood diseases are the most common causes of Budd-Chiari syndrome]. 251 87

Although cases of hepatic vein thrombosis (Budd Chiari Syndrome) in oral contraceptive (OC) users have been reported in the literature, the association has not been definitively established. Hepatic vein thrombosis, an uncommon disorder, presents with right upper quadrant abdominal pain, hepatomegaly, and ascites. Diagnostic procedures include hepatic scintiscans, ultrasonography, computerized tomography and magnetic resonance imaging, determination of intrahepatic pressure, liver biopsy, and inferior vena cava and hepatic venography. Hepatic vein thrombosis may develop without an apparent underlying cause or as a complication of an illness known to be associated with vascular thromboses such as polycythemia rubra vera or paroxysmal nocturnal hemoglobinuria. In relation to the large numbers of women taking OCs, there have been very few cases of hepatic vein thrombosis. Evidence linking OC use to the development of hepatic adenomas is far more convincing. In a multicenter case-control study of 33 cases of hepatic vein thrombosis in women 15-45 years of age, each of whom was matched to 3-4 controls, the relative risk of hepatic vein thrombosis in OC users compared with nonusers was 2.37 (p 0.02). It was noted that the 1 patient with paroxysmal nocturnal hemoglobinuria, 5 of 12 patients with overt primary myeloproliferative disorder, and 7 of 8 patients with a forme fruste of a myeloproliferative disorder were OC users, suggesting that OCs--through their thrombogenic action--augmented the thrombotic tendency of the underlying condition. The objectives of therapy in hepatic vein thrombosis are to relieve the hepatic congestion and prevent further clot formation. The majority of patients die within 3 years of diagnosis.
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PMID:Hepatic vein thrombosis (Budd Chiari syndrome): possible association with the use of oral contraceptives. 329 14

The association of erythrocyte disorders and thrombosis in polycythemia vera, paroxysmal nocturnal hemoglobinuria (PNH) and sickle cell anemia suggest that erythrocyte abnormality, could be involved in the genesis of thrombosis. A significant correlation between the hematocrit level and the incidence of thrombosis has been reported indicating the role of blood viscosity, but did to exclude an intrinsic red cell. Thrombotic episodes in sickle cell anemia might be explained by a decreased deformability and an increased adhesion of red cells to endothelium. The incidence of thrombosis is of 40 p. 100 in patient with PNH with a frequent sus-hepatic localization. In addition to abnormal erythrocyte adhesion, abnormal membrane lipid distribution was observed in PNH, suggesting that they could participate in the pathophysiology of thrombosis.
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PMID:[Role of erythrocytes in thromboembolism pathology]. 381 82

Burst-promoting activity (BPA) in the sera of patients with various types of anemia and polycythemia was compared with that of normal subjects by an in vitro method using mouse bone marrow cells. The control culture contained normal human AB serum instead of sample materials. Results were expressed as a percentage of burst numbers in control cultures. Serum erythropoietin (Epo) levels were determined by a radioimmunoassay. Serum BPA in patients with aplastic anemia (155.4 +/- 56.7%, mean +/- SD) was significantly higher than that in normal subjects (112.1 +/- 29.1%, Wilcoxon's rank sum test, P less than 0.05). However, serum BPA in patients with uremic anemia (122.2 +/- 26.5%), polycythemia vera (101.9 +/- 19.5%) and stress polycythemia (115.5 +/- 25.6%) was not significantly different from normal subjects. There was a correlation between serum BPA and Epo titers in patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria (r = 0.81, t test, P less than 0.001).
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PMID:Burst-promoting activity in anemia and polycythemia. 395 12

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.
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PMID:Hepatic vein thrombosis (Budd-Chiari syndrome). 669 73

Venous thromboembolic diseases are of major importance with respect to morbidity and mortality. Therefore, efficient prophylaxis is essential. Indication for thromboprophylaxis has to be made individually: In high risk situations, especially in orthopedic surgery, every patient should receive medical prophylaxis, e.g. with heparin, in addition to other preventive measures such as the wearing of elastic stockings or physiotherapy until full mobilization. For high-risk patients having a history of recurrent venous thromboembolism or which are suffering from a thrombogenic disease (e.g. myeloproliferative disorder, especially polycythemia vera, paroxysmal nocturnal hemoglobinuria, systemic lupus erythematosus, homocystinuria) or a hereditary thrombophilia (e.g. deficiency of antithrombin III, protein S, protein C or APC resistance), prophylactic measures should be more generally applied. In these patients, risk factors (e.g. oral contraceptive medication) or risk situations (e.g. long-distance travelling by car or airplane) have to be avoided whenever possible. In inevitable risk situations (e.g. perioperative or peripartal period) prophylaxis is mandatory. It is generally limited to the period of elevated thrombogenic risk and is often effected by application of a low molecular weight heparin. Patients with a history of recurrent thromboembolic events despite elimination of all avoidable risk factors should get a lifelong prophylaxis, usually with oral anticoagulants.
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PMID:[Prevention of venous thromboembolism--in whom, when and how?]. 783 22


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