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| {{Infobox_Disease | | | __NOTOC__ |
| Name = {{PAGENAME}} |
| | {{Polycythemia vera}} |
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| | {{CMG}}; {{AE}} {{IO}}, {{MJK}}, {{shyam}} |
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| DiseasesDB = |
| | {{SK}} Erythemia; Osler-Vaquez disease; polycythemia rubra vera; Primary polycythemia; Vaques-Osler arythremia; Vaquez' polycythaemia; Vaquez-Osler disease |
| ICD10 = {{ICD10|D|45||d|37}} |
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| ICD9 = {{ICD9|238.4}} |
| | ==[[Polycythemia vera overview|Overview]]== |
| ICDO = 9950/3 |
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| MeshID = D011087 |
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| {{CMG}}
| | ==[[Polycythemia vera historical perspective|Historical Perspective]]== |
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| {{Polycythemia vera}}
| | ==[[Polycythemia vera classification|Classification]]== |
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| ==[[Polycythemia vera overview|Overview]]== | | ==[[Polycythemia vera pathophysiology|Pathophysiology]]== |
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| ==[[Polycythemia vera epidemiology and demographics|Epidemiology & Demographics]]== | | ==[[Polycythemia vera differential diagnosis|Differentiating Polycythemia vera from other Diseases]]== |
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| '''Polycythemia vera''' occurs in all age groups (including children),<ref>{{cite journal | author = Passamonti F, Malabarba L, Orlandi E, Baratè C, Canevari A, Brusamolino E, Bonfichi M, Arcaini L, Caberlon S, Pascutto C, Lazzarino M | title = Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia. | journal = Haematologica | volume = 88 | issue = 1 | pages = 13-8 | year = 2003 | id = PMID 12551821}}</ref> although the incidence increases with age. One study found the median age at diagnosis to be 60 years,<ref name="pvsg">Berlin, NI. (1975). "Diagnosis and classification of polycythemias". ''Semin Hematol'' '''12''': 339.</ref> while a study in Olmsted County, Minnesota found that the highest incidence was in people aged 70–79 years.<ref name="olmsted">{{cite journal | author = Anía B, Suman V, Sobell J, Codd M, Silverstein M, Melton L | title = Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989. | journal = Am J Hematol | volume = 47 | issue = 2 | pages = 89-93 | year = 1994 | id = PMID 8092146}}</ref> The overall incidence in the Minnesota population was 1.9 per 100,000 person-years, and the disease was more common in men than women.<ref name="olmsted"/>
| | ==[[Polycythemia vera epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==[[Polycythemia vera history and symptoms|History & Symptoms]]== | | ==[[Polycythemia vera risk factors|Risk Factors]]== |
| Patients with polycythemia vera are often [[asymptomatic]]. A classic symptom of polycythemia vera is [[pruritis|generalized itching]], particularly after exposure to warm water, which may be due to abnormal [[histamine]] release<ref>{{cite journal | author = Steinman H, Kobza-Black A, Lotti T, Brunetti L, Panconesi E, Greaves M | title = Polycythaemia rubra vera and water-induced pruritus: blood histamine levels and cutaneous fibrinolytic activity before and after water challenge. | journal = Br J Dermatol | volume = 116 | issue = 3 | pages = 329-33 | year = 1987 | id = PMID 3567071}}</ref><ref>{{cite journal | author = Jackson N, Burt D, Crocker J, Boughton B | title = Skin mast cells in polycythaemia vera: relationship to the pathogenesis and treatment of pruritus. | journal = Br J Dermatol | volume = 116 | issue = 1 | pages = 21-9 | year = 1987 | id = PMID 3814512}}</ref> or [[prostaglandin]] production.<ref>{{cite journal | author = Fjellner B, Hägermark O | title = Pruritus in polycythemia vera: treatment with aspirin and possibility of platelet involvement. | journal = Acta Derm Venereol | volume = 59 | issue = 6 | pages = 505-12 | year = 1979 | id = PMID 94209}}</ref> Such itching is present in approximately 40% of patients with polycythemia vera.<ref name="pvsg"/> [[Gout|Gouty arthritis]] may be present in up to 20% of patients.<ref name="pvsg"/> [[Peptic ulcer disease]] is also common in patients with polycythemia vera; the reasons for this are unclear, but may be related to an increased susceptibility to infection with the ulcer-causing bacterium ''[[Helicobacter pylori|H. pylori]]''.<ref>{{cite journal | author = Torgano G, Mandelli C, Massaro P, Abbiati C, Ponzetto A, Bertinieri G, Bogetto S, Terruzzi E, de Franchis R | title = Gastroduodenal lesions in polycythaemia vera: frequency and role of Helicobacter pylori. | journal = Br J Haematol | volume = 117 | issue = 1 | pages = 198-202 | year = 2002 | id = PMID 11918555}}</ref>
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| A rare but classic symptom of polycythemia vera (and the related myeloproliferative disease [[essential thrombocythemia]]) is [[erythromelalgia]].<ref>{{cite journal | author = van Genderen P, Michiels J | title = Erythromelalgia: a pathognomonic microvascular thrombotic complication in essential thrombocythemia and polycythemia vera. | journal = Semin Thromb Hemost | volume = 23 | issue = 4 | pages = 357-63 | year = 1997 | id = PMID 9263352}}</ref> This is a sudden, severe burning pain in the hands or feet, usually accompanied by a reddish or [[cyanosis|bluish]] coloration of the skin. Erythromelalgia is caused by an increased platelet count or increased platelet "stickiness", resulting in the formation of tiny blood clots in the vessels of the extremity; it responds rapidly to treatment with [[aspirin]].<ref>{{cite journal | author = Michiels J | title = Erythromelalgia and vascular complications in polycythemia vera. | journal = Semin Thromb Hemost | volume = 23 | issue = 5 | pages = 441-54 | year = 1997 | id = PMID 9387203}}</ref><ref>{{cite journal | author = Landolfi R, Ciabattoni G, Patrignani P, Castellana M, Pogliani E, Bizzi B, Patrono C | title = Increased thromboxane biosynthesis in patients with polycythemia vera: evidence for aspirin-suppressible platelet activation in vivo. | journal = Blood | volume = 80 | issue = 8 | pages = 1965-71 | year = 1992 | id = PMID 1327286}}</ref>
| | ==[[Polycythemia vera screening|Screening]]== |
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| Patients with polycythemia vera are prone to the development of blood clots ([[thrombosis]]). A major thrombotic complication (e.g. [[myocardial infarction|heart attack]], [[stroke]], [[deep venous thrombosis]], or [[Budd-Chiari syndrome]]) may sometimes be the first symptom or indication that a person has polycythemia vera.
| | ==[[Polycythemia vera natural history, complications, and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Diagnosis== | | ==Diagnosis== |
| ==[[Polycythemia vera laboratory tests|Lab Tests]]==
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| | [[Polycythemia vera diagnostic criteria|Diagnostic Criteria]] | [[Polycythemia vera history and symptoms|History and Symptoms]] | [[ Polycythemia vera physical examination|Physical Examination]] | [[Polycythemia vera laboratory tests|Laboratory Findings]] | [[Polycythemia vera electrocardiogram|Electrocardiogram]] | [[Polycythemia vera x-ray|X-ray]] | [[Polycythemia vera CT|CT]] | [[Polycythemia vera MRI|MRI]] | [[Polycythemia vera ultrasound|Ultrasound]] | [[Polycythemia vera other imaging findings|Other Imaging Findings]] | [[Polycythemia vera other diagnostic studies|Other Diagnostic Studies]] |
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| ==Treatment== | | ==Treatment== |
| | [[Polycythemia vera medical therapy|Medical Therapy]] | [[Polycythemia vera surgery |Surgery]] | [[Polycythemia vera primary prevention|Primary Prevention]] | [[Polycythemia vera secondary prevention|Secondary Prevention]] | [[Polycythemia vera cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Polycythemia vera future or investigational therapies|Future or Investigational Therapies]] |
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| ==[[Polycythemia vera medical therapy|Medical Therapy]]== | | ==Case Studies== |
| | [[Polycythemia vera case study one|Case #1]] |
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| ==External links==
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| * [http://members.aol.com/mpdsupport Myeloproliferative Disease Support Group, since 1994]
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| * [http://www.mpdinfo.org/ Myeloproliferative Disorders (CMPD Education Foundation)]
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| * [http://www.mpdfoundation.org MPD Foundation and Research Alliance - Register for a free newsleter]
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| ==References==
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| {{Reflist|2}}
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| {{Hematology}} | | {{Hematology}} |
| {{Hematological malignancy histology}} | | {{Hematological malignancy histology}} |
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| [[de:Polycythaemia vera]]
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| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| | [[Category:Disease]] |
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