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)

The authors report the clinical and hematological course of 20 patients with polycythemia vera complicated by acute leukemia. This was responsible for 35,8% of the 53 deaths in the series of 103 patients treated with radioactive phosphorus. It occurred on average 12 years after the first treatment. The data in these cases are compared with those in the literature. The role of ionising radiation and/or the myelo-proliferative syndrome itself in the onset of acute leukemia is discussed.
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PMID:[Acute leukemia and primary Vaquez' polycythemia. 20 cases]. 21 57

A group of 76 polycythemia vera patients was followed prospectively with bone marrow examinations at regular intervals. Six terminated in acute leukemia, preceded by an indolent preleukemic phase succeeded by a very fulminant leukemia. At autopsy, all patients had extramedullary hematopoiesis. In one of them, only the bone marrow was leukemic, suggesting that it was the primary site of this transformation. In three with leukemic involvement of nonhematopoietic organs, the infiltrate consisted of myelo- or myelomonoblasts and normoblasts. The preleukemic phase was characterized by a rapidly evolving spontaneous pancytopenia, with a drop from polycythemic blood counts within 2 mo. In the bone marrow, megaloblastoid erythropoiesis with ring sideroblasts was prominent. A similar preleukemic phase evolving in other potentially leukemic conditions, has been described. Therefore, in patients at risk, the appearance of ring sideroblasts may be regarded as an early indicator of imminent acute leukemic transformation.
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PMID:Preleukemia and leukemia in polycythemia vera. 124 21

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

Plasma fibronectin concentration was determined by electroimmunodiffusion and laser nephelometry in 40 healthy persons and 321 patients with myelo- and lymphoproliferative diseases and other malignancies. Decreased fibronectin concentrations were found in patients with leukemia, Hodgkin's and non-Hodgkin's lymphomas, myelofibrosis, polycythaemia rubra vera and angioimmunoblastic lymphadenopathy. Elevated fibronectin level was detected in patients with multiple myeloma. In patients with cancer of the lung, stomach and colon, fibronectin level was found in the normal range. Decreased fibronectin concentration was observed in patients with cancer of the breast and prostate. Lower plasma fibronectin concentrations were detected in all groups of patients with infectious septical complications as compared to the patients without infections.
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PMID:[Determination of plasma fibronectin in myeloproliferative and lymphoproliferative diseases and other malignancies]. 308 43

The vascular risk factors of patients with polycythemia rubra vera was assessed by reviewing the results of international therapeutic studies. The risk factors were: an age over 60, previous vascular problems, the quality of follow-up (maintenance of hematocrit below 50 p. cent and platelet count below (600.10(9)/l). The treatment is one of the most important factors: the risk of vascular complications was three times greater in patients treated by venesection, al other factors being equal. This counter-balanced the higher risk of leukemia in patients treated by myelo-depression. The use of platelet anti-agregant drugs remains controversial. Recent results question their efficacy in preventing thrombosis and emphasise the risk of hemorrhage. The results of platelet factor 4 (PF4) and beta-thromboglobin (beta TG) levels provide further information for judging the vascular risk of these patients, and may, in prospective studies, give a better assessment of the efficacy of platelet anti-agregant drugs in vivo.
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PMID:[Vascular complications of polycythemia]. 622 77

Content and composition of glycosaminoglycans (GAG) was studied in leukocytes of 30 healthy persons and of patients with different types of myeloproliferative disorders: 32 patients with chronic myeloid leukemia (CML), 10 patients with polycythemia vera (PV), 5 patients with idiopathic myeloid fibrosis (IMF) and 10 patients with acute myelo blast leukemia (AML). The total content of GAG in healthy persons was 97.7 +/- 3.9 micrograms of uronic acid per 100 mg of dry cells, in CML and IMF it was 2-4-fold increased, in PV it was elevated by 20%, in AML it was diminished 3-5-fold. The elevated level of GAG in CML and IMF was due to presence of basophils and considerable quantity of immature cell forms: promyelocytes and myelocytes. In these cells changes in GAGs composition and their properties were shown: an increase of chondroitins deficient in sulfate groups and of heparan sulfate content, especially in basophils. The observed changes in GAG content and composition may be important for biological activity of the leukemic cells.
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PMID:[Glycosaminoglycans in leukocytes in myeloproliferative disorders]. 659 25

An increased platelet number in blood depends on a limited spectrum of causes, which aren't always simple to identify. Secondary thrombocytosis is a reactive process in relation with acute or chronic inflammatory diseases, or asplenia. The infrequent inherited thrombocytoses disorders are suspected when similar cases are observed in the same family. However, the most frequent causes of chronic thrombocytosis in adults are the so-called chronic myeloproliferative syndromes (chronic myelocytic leukaemia, polycythemia vera, primary myelofibrosis, essential thrombocytemia), and to a lesser extent, myelodysplastic syndromes. In the course of these disorders, thrombocytosis is often the first recognized abnormality. Chronic myelocytic leukaemia is easily diagnosed owing to the presence of either the Philadelphia chromosome or the BCR-ABL fusion gene product. The next step still relies upon a distinction according to the PVSG or the WHO criteria of Polycythemia Vera (PV) and Idiopathic myelo fibrosis (IMF) to finally confirm genuine Essential Thrombocythemia (ET). The recent description of the V617F mutation of JAK2 in 90% of PV patients, 43 to 67% with IMF and 50% of ET diagnosed according to either the PVSG or the WHO criteria is a definite characteristic of clonality now accessible in haematology practice. However, this mutation is neither specific nor constant in any of the Philadelphia negative myeloproliferative disorders, which outlines the importance of the WHO criteria of megakaryocytic abnormalities on bone marrow biopsy as the hallmark of Ph negative MPDs. The exclusion of PV and of IMF, including pre fibrotic and early fibrotic forms is still required for the diagnosis of "true" ET. Disease stratification and treatment strategy are targeted on the evaluation and prevention of vascular complications. Acute leukaemia or myelodysplasia, and other clonal progressions like myelofibrotic transformation, are infrequent and delayed events. However, according to the present data, the risk of fibrotic progression or of leukaemic transformation is not related to the mutation status of ET patients.
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PMID:[Essential thrombocythemia. Contribution of the V617F JAK2 mutation to the pathophysiology, diagnosis and outcome]. 1807 52

Polycythemia vera (PV) is a rare progressive myelo-proliferative cancer with significant symptom burden. Patients with PV often experience symptoms that adversely affect quality of life, work productivity, and functional status. Oncology nurses are well suited to assess for symptom burden and to provide educational interventions that support patients and their families.
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PMID:Polycythemia Vera: Symptom Burden, Oncology Nurse Considerations, and Patient Education. 3294 92