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)

Thirty-six patients with chronic myeloproliferative disorders (CMPD) were studied as regards blood coagulation and fibrinolysis. These studies revealed various mild abnormalities: activated thromboplastin time (APTT) tended to prolong and the level of factor V decreased significantly. In several cases, the levels of D-dimer, thrombin-antithrombin III complex and plasmin-alpha 2-plasmin inhibitor complex were elevated compared to normal. These results suggest that abnormal coagulation system in the patients with CMPD is related to low grade disseminated intravascular coagulation. Many coagulation factors did not correlate with peripheral blood cell counts. Two patients with polycythemia vera were evaluated for several abnormalities of the coagulation system before and during treatment. Coagulation abnormalities persisted after hematologic control had been achieved. Our results suggest that patients with CMPD have a chronic state of abnormal blood coagulation system even after normalization of blood cell counts.
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PMID:[Abnormal blood coagulation and fibrinolysis in chronic myeloproliferative disorders]. 187 Feb 70

Phenylhydrazine (PHZ) is a hemolytic agent which has been used in the treatment of polycythemia vera. Recent studies performed in our laboratory have indicated that the PHZ-induced anemia is immuno-hemolytic in etiology, and a prolonged bleeding time was present in some of the rats chronically treated with PHZ. The nature of this bleeding tendency was explored in the present experiment. PHZ was administered to rats once a week for a six week period. During this time, the animals were monitored for prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen concentration, and individual coagulation factor levels as well as routine plasma chemistries and blood cell counts. In addition, radioimmunoassays (RIA) for prostacyclin, a platelet aggregation inhibitor, and prostaglandin (PG) E2 were performed. PHZ-treated animals displayed a significant elevation in both PT and APTT when compared with saline injected controls, although plasma fibrinogen levels were not appreciably altered. Further tests revealed a PHZ-induced decrease in prothrombin and factor V levels. In addition, a significant increase in plasma serum glutamate oxaloacetate transaminase (SGOT), lactate dehydrogenase (LDH), and alkaline phosphatase levels was observed as well as a diminution in cholesterol and triglycerides following PHZ administration. PHZ treatment also induced an elevation in prostacyclin levels and transient thrombocytopenia. These findings indicate that several factors may contribute to the prolonged bleeding time in PHZ-treated rats including a drug induced thrombocytopenia possibly associated with enhanced synthesis of autologous immunoglobulin G (IgG) against the senescent red cell antigen, and diminished synthesis of vitamin K-dependent coagulation factors which may be mediated by reduced vitamin K uptake by the hypo-cholesterolemic subjects.
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PMID:Hemostatic alterations associated with phenylhydrazine-induced anemia in the rat. 262 15

Blood coagulation and fibrinolysis were studied in 65 patients with chronic myeloproliferative disorders (MPD). They consisted of 28 patients with chronic granulocytic leukemia (CGL) in chronic phase, 7 with CGL in blast crisis, 9 with polycythemia vera (PV), 13 with primary thrombocythemia (PTh) and 8 with primary myelofibrosis (MF). Hemorrhagic and thrombotic complications were observed in 19 and 8 patients, respectively. Activated partial thromboplastin time and prothrombin time were prolonged in many patients. Low factor II levels were observed in some CGL patients. Factor V was decreased in CGL patients in chronic phase and in PV patients. Fibrinogen was either normal or increased in most patients, but an elevation of fibrin/fibrinogen degradation products (FDP) was found in some patients. The VIIIR: Ag/VIII:C ratio was increased in CGL patients in blast crisis, in PV patients and in PTh patients. Antithrombin III and plasminogen were below normal in some patients. Most patients showed a decrease in alpha 2-plasmin inhibitor. These findings suggest that blood coagulation and fibrinolysis are involved in the pathogenesis of the thrombotic and hemorrhagic complications in these patients. Chronic low-grade intravascular coagulation might be present in some patients with MPD.
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PMID:Profile of blood coagulation and fibrinolysis in chronic myeloproliferative disorders. 695 82

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.
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PMID:[Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome]. 1552 39

Hemostatic disorders are a major clinical problem in patients with myeloproliferative neoplasms (MPNs) and they are the second most common cause of death in MPN patients, after infections. The aim of this study was to assess the fibrinolytic potential of the blood of patients with MPNs. The study involved 112 patients with MPNs diagnosed at the Hematology Clinic Dr J. Biziel University Hospital No. 2 in Bydgoszcz, Poland. The study group included 63 patients with essential thrombocythemia, 29 with polycythemia vera, 11 with chronic myelogenous leukemia (CML) and nine with primary myelofibrosis. The control group consisted of 25 healthy volunteers who were age and sex-matched. The following parameters were determined: concentration of tissue plasminogen activator antigen (t-PA:Ag), plasminogen activator inhibitor type 1 antigen concentration (PAI-1:Ag), D-dimer, thrombin-antithrombin complexes, fibrinogen, activated partial thromboplastin time and international normalized ratio. The study showed significantly increased t-PA:Ag, PAI-1:Ag and D-dimer levels in patients with MPNs. Moreover, we found increased concentrations of thrombin-antithrombin complexes and fibrinogen, as well as elevated platelet counts. Detailed analysis revealed that t-PA:Ag concentration was elevated in patients with essential thrombocythemia, CML and polycythemia vera. Concentration of PAI-1:Ag was increased in patients with essential thrombocythemia and polycythemia vera; D-dimer was significantly higher in essential thrombocythemia, polycythemia vera, CML and primary myelofibrosis patients. Increased concentrations of t-PA:Ag and D-dimer indicate secondary activation of the fibrinolytic system in patients with MPNs. Elevated levels of PAI-1 in MPN patients may result from its increased production by elevated number of activated platelets and vascular endothelial damage. PAI-1 by having an inhibitory effect on fibrinolysis manifests its procoagulant activity.
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PMID:Selected parameters of hemostasis in patients with myeloproliferative neoplasms. 2450 38

A 52-year-old woman was admitted to the hospital three times in a span of 5 years in hypovolemic shock because of spontaneous and massive bleeding in the pleural and abdominal cavity. Blood tests revealed a high number of blood cells, and bone marrow smears showed trilineage myeloproliferation. Serum erythropoietin level was decreased. Analysis revealed a V617F mutation in the JAK2 protein. Her activated partial thromboplastin time was slightly prolonged, the ratio between von Willebrand factor (vWF) propeptide and vWF antigen was in the normal range, but the ratio between vWF and ristocetin cofactor was decreased dramatically. Further investigation revealed the absence of large and intermediate vWF-multimers. She was diagnosed with polycythemia vera with acquired von Willebrand syndrome. The bleeding was stopped using a transfusion of freshly thawed plasma and cryoprecipitate.
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PMID:Acquired von Willebrand syndrome in a case of polycythemia vera resulting in recurrent and massive bleeding events in the pleural and abdominal cavity. 2521 16

Bilateral adrenal hemorrhage (BAH) is a rare complication typically seen in critically ill patients, which can lead to acute adrenal insufficiency and death unless it is recognized promptly and treated appropriately. We describe the case of a 64-year-old man with polycythemia vera found to be unresponsive with fever, hypotension, tachycardia, and hypoglycemia. Electrocardiogram showed ST-elevation with elevated troponin, hemoglobin, prothrombin time, and partial thromboplastin time. He required aggressive ventilator and vasopressor support. Despite primary coronary intervention, he remained hypotensive. Random cortisol level was low. He received stress dose hydrocortisone with immediate hemodynamic stability. BAH was highly suspected and was confirmed by non-contrast abdominal computed tomography. Prompt recognition and timely initiated treatment remain crucial to impact the mortality associated with acute adrenal insufficiency.
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PMID:Bilateral adrenal hemorrhage in polycythemia vera. 2760 33