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

The clinical characteristics of aquagenic pruritus (AP) based on a series of thirty-six patients are presented. AP is characterized by the development of severe, prickling-like skin discomfort that is without observable skin lesions and that is evoked by contact with water at any temperature. Other causes of pruritus associated with water contact must be excluded. In the thirty-six patients, skin discomfort developed within minutes of water contact in approximately half. In others, discomfort began 2 to 15 minutes after water exposure had ceased. The pruritus was usually generalized, lasting from 10 to 120 minutes (average, 40.6 minutes), and in 55% was associated with symptoms of acute emotional liability. There was no increased prevalence of atopy. Thirty-three percent reported a family history of water-related itching. Of fourteen patients treated with ultraviolet B phototherapy, eight (57%) noted significant relief. Of thirty-four patients, sixteen (47%) noted partial relief with oral antihistamine therapy. Patients with polycythemia rubra vera (PRV) may present with symptoms similar to those of AP, and all patients with symptoms consistent with AP should be investigated for the presence of PRV.
 ...
PMID:Aquagenic pruritus. 241 68

A 13 year old Thoroughbred gelding was presented with a history of a single episode of collapse during mild exercise. Clinical examination revealed a high packed cell volume (PCV) of 72%, a haemoglobin concentration of 24.9 g/l and 15.2 millions erythrocytes/microliters. Despite continuous intravenous infusion therapy with large volumes, the PCV never decreased to a physiological level. The animal showed a normal appetite and no signs of discomfort or syncope. Arterial blood gas values were in the normal range as well as the concentration of erythropoietin (measured by radioimmunoassay, RIA). A test for neoplasms (carcino-embryonic antigen, CEA) was negative. The liver enzymes of the animal were extremely elevated and a liver biopsy showed a severe fibrosis. Examination of sternal bone marrow aspirate revealed no abnormalities. Based on these findings, the presumptive diagnosis was "absolute polycythaemia". The animal was treated for 7 days with repeated phlebotomy. During this time, the PCV never decreased below 50%, despite no obvious signs of discomfort from the animal. Because of the poor prognosis based on the liver biopsy result, the animal was euthanized 11 days after hospitalization. Post mortem findings were: a granular cell myoblastoma with a diameter of approximately 5 cm in the lungs, severe fibrosis of the liver, mild acute tubular nephrosis in the kidneys, activation of the erythropoietic cells in the bone marrow and thrombosis of the abdominal aorta. The possibility of secondary polycythaemia due to the lung neoplasia was not entirely excluded, but considered to be unlikely. Therefore, the definite diagnosis was polycythemia vera.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Case report: polycythemia in a horse]. 756 45

Myelofibrosis with myeloid metaplasia (MMM) is a collective term that describes the related disorders AMM, PPMM, and PTMM. The chronic myeloid disorders include chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, and agnogenic myeloid metaplasia (myelofibrosis). These disorders display varying propensities for pathologic enlargement of the spleen which can lead to mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Splenectomy has been found to be of little benefit in the early stages of chronic myeloid leukemia. Similarly, the benefit of splenectomy in advanced cases is limited to symptomatic palliation and treatment of delayed engraftment after allogeneic bone marrow transplantation. Although polycythemia vera and essential thrombocythemia are also characterized by splenomegaly, splenectomy is not considered a therapeutic option in the absence of transformation of the disease into myelofibrosis with myeloid metaplasia. Splenectomy has been studied most in myelofibrosis with myeloid metaplasia. Although there is no clear survival advantage to splenectomy in this disorder, the surgical procedure can result in substantial palliation of mechanical discomfort, hypercatabolic symptoms, portal hypertension, and anemia. However, the procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern.
 ...
PMID:Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia. 1098 48

Polycythemia vera in patients undergoing cardiac surgery is clinically rare. A 65-year-old man with polycythemia vera was admitted with effort-related chest discomfort. We planned coronary artery bypass grafting for left anterior descending artery and obtuse marginal branch stenosis, using bilateral internal thoracic arteries, with perioperative prophylactic management to avoid thromboembolism. His internal thoracic arterial grafts occluded during and after surgery due to thrombus, and ST-elevation myocardial infarction developed, which needed a percutaneous coronary intervention. This case suggests that optimal management methods should be studied further to contribute to better patient outcomes in this condition.
...
PMID:Acute coronary artery bypass graft failure in a patient with polycythemia vera. 2519 84

BCR-ABL-negative myeloproliferative neoplasms include primary myelofibrosis, polycythemia vera, and essential thrombocythemia. Clonal stem cell proliferation and dysregulated JAK/STAT molecular pathways characterize these hematologic malignancies. Symptoms experienced by patients are heterogeneous including excessive and disabling fatigue, early satiety, anorexia, pruritus, bone pain, night sweats, cachexia, abdominal pain and discomfort, and cognitive complaints. Patients also experience impaired quality of life along with decreased overall survival. New targeted drug therapies, including JAK2 inhibitors, have demonstrated remarkable success in alleviating the myeloproliferative neoplasm (MPN) symptomatic burden, reducing splenomegaly and improving quality of life while offering overall survival benefit. Within the USA, FDA approval has only been granted to use JAK2 inhibitors in intermediate- to high-risk myelofibrosis. However, given that low-prognostic-scoring patients have been shown to have considerable symptomology, there is a possibility that lower-risk patients may benefit from therapy. More than ever, the need for accurate MPN symptom burden assessment and subsequent addition of targeted therapies is critical in the treatment of MPNs. This article discusses the role of MPN symptom burden and quality of life as therapeutic targets in the context of recent MPN clinical advances.
 ...
PMID:Quality of life in MPN comes of age as a therapeutic target. 2526 10

The myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and myelofibrosis, are distinguished by their debilitating symptom profiles, life-threatening complications and profound impact on quality of life. The role gender plays in the symptomatology of myeloproliferative neoplasms remains under-investigated. In this study we evaluated how gender relates to patients' characteristics, disease complications and overall symptom expression. A total of 2,006 patients (polycythemia vera=711, essential thrombocythemia=830, myelofibrosis=460, unknown=5) were prospectively evaluated, with patients completing the Myeloproliferative Neoplasm-Symptom Assessment Form and Brief Fatigue Inventory Patient Reported Outcome tools. Information on the individual patients' characteristics, disease complications and laboratory data was collected. Consistent with known literature, most female patients were more likely to have essential thrombocythemia (48.6% versus 33.0%; P<0.001) and most male patients were more likely to have polycythemia vera (41.8% versus 30.3%; P<0.001). The rate of thrombocytopenia was higher among males than females (13.9% versus 8.2%; P<0.001) and males also had greater red-blood cell transfusion requirements (7.3% versus 4.9%; P=0.02) with shorter mean disease duration (6.4 versus 7.2 years, P=0.03). Despite there being no statistical differences in risk scores, receipt of most therapies or prior complications (hemorrhage, thrombosis), females had more severe and more frequent symptoms for most individual symptoms, along with overall total symptom score (22.8 versus 20.3; P<0.001). Females had particularly high scores for abdominal-related symptoms (abdominal pain/discomfort) and microvascular symptoms (headache, fatigue, insomnia, concentration difficulties, dizziness; all P<0.01). Despite complaining of more severe symptom burden, females had similar quality of life scores to those of males. The results of this study suggest that gender contributes to the heterogeneity of myeloproliferative neoplasms by influencing phenotypic profiles and symptom expression.
 ...
PMID:Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: an analysis by the MPN QOL International Working Group. 2754 Jan 37