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 haematological, biochemical and clinical features of six patients with polycythaemia after renal transplantation were studied. The patients had an absolute increase in red cell mass, but normal plasma and whole blood volumes. Primary proliferative polycythaemia was excluded. Polycythaemia developed within one year of transplantation and persisted for 3-7 years. Chronic rejection, renal artery stenosis, severe hypertension and corticosteroid therapy were probably not the cause of the polycythaemia. There were no occlusive vascular lesions during the observation period and venesections were generally not required. The polycythaemia is probably the result of the cumulative production of erythropoietin by the donor and recipient kidneys.
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PMID:Polycythaemia after renal transplantation. 331 62

Hypertension and polycythaemia rubra vera commonly occur in the same patient. This is regarded as coincidence and the hypertension does not respond to correction of polycythaemia. We report a case of renal artery stenosis occurring simultaneously with polycythaemia rubra vera and suggest that renovascular occlusive disease should be excluded in such patients with refractory hypertension.
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PMID:Reno-vascular disease in polycythaemia rubra vera. 663 46

A case of posttransplant erythrocytosis in a 51-year-old diabetic man is described. This problem, which can occur in 5 to 15% of renal transplant patients, can result from a contracted plasma volume (diuretics, pressure natriuresis, or glycosuria) or from a true elevation in red blood cell mass. Once the diagnosis of true erythrocytosis is made by a radiolabeled red blood cell mass study, secondary causes such as hypoxia, liver disease, polycythemia rubra vera, renal artery stenosis, and cystic kidney disease should be excluded. Posttransplant erythrocytosis has only been observed in renal transplant recipients and appears to be more frequent with cyclosporine compared with azathioprine therapy. An inappropriately high level of erythropoietin has been described in some, but not all patients, suggesting stimulation of erythropoietin production as the mechanism. Posttransplant erythrocytosis can be associated with an increased incidence of thrombotic events. The presence of this potential complication has prompted intervention to maintain the hematocrit below 50 to 55%. Measures such as discontinuation of diuretics as well as better control of blood pressure and plasma glucose should be used to facilitate the correction of extracellular volume contraction. Phlebotomy has been the most accepted intervention to intermittently lower the hematocrit when needed, but this can lead to iron deficiency. Newer therapeutic modalities are now being used to treat the problem medically. Theophylline, which reduces adenosine-mediated erythropoietin synthesis, is effective but may be associated with side effects.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Posttransplant erythrocytosis: case report and review of newer treatment modalities. 831 81

Polycythaemia vera represents a rare chronic myeloproliferative neoplasm characterized by an increased thrombotic risk. Previous case reports have documented a link between primary or secondary polycythemia and the presence of renal artery stenosis and renovascular hypertension. Herein, we report an exceptional case of renal artery restenosis leading to uncontrolled hypertension in a patient with PV and high haematocrit levels. A 52-year-old female patient with a history of polycythaemia vera under treatment with hydroxyurea and phlebotomy presented in our outpatient clinic with newly diagnosed hypertension caused by left renal artery stenosis. Six months after stenting, patient returned for a follow-up visit due to uncontrolled hypertension and high haematocrit levels. Total restenosis of the left renal artery was found. Patient received optical medical treatment and was prescribed to higher doses of hydroxyurea by her treating haematologist. Since then, blood pressure and Hct levels remain adequately controlled. As described by earlier case reports, renal artery stenosis, hypertension and polycythemia often coexist. However, renovascular hypertension may not only lead to secondary erythrocytosis but also be a thrombotic complication of primary erythrocytosis. Thus, patients with polycythaemia vera should be carefully evaluated and optimally managed when hypertension or impaired renal function coexist.
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PMID:An exceptional case of renal artery restenosis in a patient with polycythaemia vera. 2499 47

Clinical syndrome of refractory secondary hypertension, renal artery stenosis and secondary erythrocytosis could occur in the same patient. We report a rare case of refractory secondary hypertension, renal artery stenosis and primary erythrocytosis as an expression of polycythaemia rubra vera (PV) and suggest that erythrocytosis in a hypertensive renovascular occlusive disease may be primary due to underlying PV. This clinical syndrome should be excluded in such patients with refractory hypertension.
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PMID:A rare clinical syndrome of refractory secondary hypertension, renal artery stenosis and erythrocytosis. 2598 50

Myeloproliferative neoplasms (MPNs) with Janus kinase 2 (JAK2) mutation are associated with a high risk for occlusive vascular diseases. We report 2 cases of renovascular hypertension associated with JAK2 V617F mutation-positive MPNs and provide a literature review. In Case 1, a 63-year-old woman had resistant hypertension, massive proteinuria, and erythrocytosis. Evaluations revealed right renal artery stenosis causing renovascular hypertension and polycythemia vera with JAK2 V617F mutation. Renin-angiotensin system inhibitors and subsequent angioplasty controlled the blood pressure and the proteinuria resolved. In Case 2, a 74-year-old woman had resistant hypertension and thrombocytosis. Evaluations confirmed left renal artery stenosis and essential thrombocythemia with JAK2 V617F. Angioplasty cured the hypertension. A literature review of 18 cases revealed the following as the most common characteristics of MPN-associated renovascular hypertension: manifests primarily in women; is associated with untreated polycythemia vera and essential thrombocythemia, concomitant leukocytosis, and JAK2 mutation positivity; and is responsive to angioplasty. This report demonstrates that JAK2 mutation-positive MPNs are a less common but important underlying cause of adult renovascular hypertension.
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PMID:Renovascular hypertension associated with JAK2 V617F positive myeloproliferative neoplasms treated with angioplasty: 2 cases and literature review. 2965 57