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)

Polycythemia vera (PV) is characterized by low serum total cholesterol despite its association with vascular events such as myocardial and cerebral infarction. Serum cholesterol level has not been used as a diagnostic criterion for PV since the 2008 revision of the WHO classification. Therefore, we revisited the relationship between serum lipid profile, including total cholesterol level, and erythrocytosis. The medical records of 34 erythrocytosis patients (hemoglobin : men, > 18.5 g/dL ; women, > 16.5 g/dL) collected between August 2005 and December 2011 were reviewed for age, gender, and lipid profiles. The diagnoses of PV and non-PV erythrocytosis were confirmed and the in vitro efflux of cholesterol into plasma in whole blood examined. The serum levels of total cholesterol, low-density-lipoprotein cholesterol (LDL-Ch), and apolipoproteins A1 and B were lower in PV than in non-PV patients. The in vitro release of cholesterol into the plasma was greater in PV patients than in non-PV and non-polycythemic subjects. Serum total cholesterol, LDL-Ch, and apolipoproteins A1 and B levels are lower in patients with PV than in those with non-PV erythrocytosis. The hypocholesterolemia associated with PV may be attributable to the sequestration of circulating cholesterol into the increased number of erythrocytes.
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PMID:Hypocholesterolemia in patients with polycythemia vera. 2303 23

The most common neurologic manifestations of polycythemia vera (PV) are cerebral infarction and transient ischemic attacks, while cerebral hemorrhage or intracranial dissection has been rarely associated with PV. Here we report the first case of a 59-year-old patient with intracranial supraclinoid internal carotid artery (ICA) dissection causing cerebral infarction and concomitant subarachnoid hemorrhage due to pseudoaneurysm rupture as clinical onset of PV. This case report discusses the possible mechanism and treatment of this extremely rare condition.
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PMID:Concurrent Ruptured Pseudoaneurysm of the Internal Carotid Artery and Cerebral Infarction as an Initial Manifestation of Polycythemia Vera. 2636 30

Among all types of myeloproliferative neoplasms (MPNs), polycythemia vera (PV) and essential thrombocythemia (ET) require careful management of both thrombosis and hemostasis. One recent concern associated with MPNs is the JAK2 mutation (V617F). This mutation is essential for MPN pathology, but it has also garnered attention for its association with thrombosis. Several studies have reported the mechanisms underlying the onset of thrombosis in both PV and ET and have also discussed the association between JAK2 mutations and thrombotic tendencies. Arterial thrombosis is a common clinical symptom that is associated with the diagnosis and course of both PV and ET. Particularly, cerebral infarction has been identified as the leading cause of death in patients with untreated PV. Notably, PV and ET are also associated with a high incidence of venous thromboembolism (VTE). The occurrence of this type of thrombosis at atypical sites, such as cerebral venous sinus thrombosis and splanchnic vein thrombosis (SVT), is not uncommon. Generally, patients with PV and ET have good life expectancy; their treatment essentially focuses on dealing with thrombosis and bleeding. Phlebotomy may be used for treating patients with PV; however, low-dose aspirin is used to prevent the onset of arterial thrombosis. For patients with a history of VTE, oral anticoagulants are commonly prescribed to prevent recurrence.
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PMID:[Thrombosis in myeloproliferative neoplasms]. 3018 3

Polycythemia vera (PV) is one of the rare causes of cerebrovascular disease, whose common manifestations in the nervous system are cerebral infarction and transient ischemic attack. A number of cases of PV patients with bleeding complicated with subdural hemorrhage or cerebral hemorrhage have been previously reported. However, sometimes PV patient with complicated cerebral hemorrhage and lower extremity venous thrombosis have been admitted to the People's Hospital of Liaoning Province. The present case study reports on a patient with acute multiple cerebral infarction with cerebral micro-hemorrhage associated with PV, who was not treated with anti-thrombosis treatment. After bloodletting treatment and hydroxyurea treatment, the patients condition was stable and they were discharged. A possible mechanism of infarction is that PV may cause abnormal proliferation of red blood cells, white blood cells and platelets in the circulation, resulting in an increase of blood viscosity and reduction of blood flow velocity. Platelet deficiency may cause abnormal blood coagulation function, which may be the reason for the blood and thrombotic diseases in patients with PV.
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PMID:Acute multiple cerebral infarction combined with cerebral microhemorrhage in Polycythemia vera: A case report. 3157 37

Thrombosis has been recognized as one of the most significant risk factors of high mortality and disability in patients with Philadelphia (Ph) chromosome negative myeloproliferative neoplasms (MPNs). However, the risk factors of thrombotic events in these patients have not been completely understood. In this study, the clinical data of 58 patients with Ph-MPNs were obtained and analyzed, including 34 cases of essential thrombocytopenia (ET), 23 thrombotic events happened in 21 (36%) patients, among which 60% (14 of 23) with cerebral infarction, 17% (4 of 23) with coronary heart disease and 23% (5 of 23) with venous thrombosis. There were no significant differences in age, sex, and blood cell count between polycythemia vera (PV) and ET patients who have experienced thrombotic events and those who have not. In ET patients, the incidence of thrombotic events in plasminogen activator inhibitor-1 (PAI-1) genotype 4G4G was significantly higher than that in genotype 4G5G and genotype 5G5G (P < .05). The incidence of thrombotic events in PV and ET patients with infection was higher than those without infection (P < .05). Using logistic regression analysis, we found that PAI-1 genotype 4G4G and infection were associated with thrombotic events (odds ratio 6.744, 95% CI: 1.195-38.056 and 15.641 95% CI: 3.327-73.522). The 4G/4G polymorphism of PAI-1 gene and infection are independent risk factors of thrombotic events in patients with Ph-MPNs. PAI-1 gene 4G4G and infection in ET and PV patients with Janus kinase 2 (JAK2) V617F mutation were shown to be high risk of thrombotic events. Therefore, clinical doctors should put more attention on PAI-1 genotype 4G4G and infection in JAK2 V617F mutated patients with Ph-MPNs to prevent the thrombosis.
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PMID:Correlation Between PAI-1 Gene 4G/5G Polymorphism and the Risk of Thrombosis in Ph Chromosome-Negative Myeloproliferative Neoplasms. 3268 89


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