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{{Polycythemia vera}}
{{Polycythemia vera}}
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==Overview==
==Overview==
'''Primary [[polycythemia]]''', often called '''[[polycythemia vera]]''' (PCV), polycythemia rubra vera (PRV), or erythremia, occurs when excess red blood cells are produced as a result of an abnormality of the [[bone marrow]]. Often, excess [[white blood cell]]s and [[platelet]]s are also produced. Polycythemia vera is classified as a [[myeloproliferative disease]].
People with polycythemia vera usually asymptomatic. Symptoms of polycythemia vera include [[headache]], [[fatigue]], and [[pruritis]].<ref name="cancer.ca">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab</ref>


==History and Symptoms==
==History and Symptoms==
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. [[Gout|Gouty arthritis]] may be present in up to 20% of patients. [[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>
Symptoms of polycythemia vera may include:<ref name="cancer.ca">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab</ref>
 
<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> .<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><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><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><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>
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>
*[[Headache]]
 
*[[Pruritis]]
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.
:*Increase after exposure to warm water
 
:*Due to [[histamine]] release or [[prostaglandin]] production
:*Present in approximately 40% of patients
*[[Tinnitis]]
*[[Blurred vision]]
*[[Malaise]]
*[[Loss of appetite]]
*[[Weight loss]]
*[[Shortness of breath]]
*[[Fatigue]]
*[[Dizziness]]
*[[Bleeding]]
:*[[Bruising]]
:*[[Epistaxis]]
:*[[Bleeding gums]]
:*[[Menorrhagia]]
:*[[Metrorrhagia]]
:*[[Petechiae]]
*[[Chest pain]]
*[[Arthralgia]]
*[[Erythromelalgia]]
:*Sudden, severe burning pain in the hands or feet
:*Accompanied by a reddish or [[cyanosis|bluish]] coloration of the skin
*[[Heart burn]]
:* Due to increased susceptibility to infection with [[Helicobacter pylori|H. pylori]]
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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{{Hematological malignancy histology}}
{{Hematological malignancy histology}}


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

People with polycythemia vera usually asymptomatic. Symptoms of polycythemia vera include headache, fatigue, and pruritis.[1]

History and Symptoms

Symptoms of polycythemia vera may include:[1] [2][3] .[4][5][6][7][8]

  • Increase after exposure to warm water
  • Due to histamine release or prostaglandin production
  • Present in approximately 40% of patients
  • Sudden, severe burning pain in the hands or feet
  • Accompanied by a reddish or bluish coloration of the skin
  • Due to increased susceptibility to infection with H. pylori

References

  1. ↑ 1.0 1.1 Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab
  2. ↑ Steinman H, Kobza-Black A, Lotti T, Brunetti L, Panconesi E, Greaves M (1987). "Polycythaemia rubra vera and water-induced pruritus: blood histamine levels and cutaneous fibrinolytic activity before and after water challenge". Br J Dermatol. 116 (3): 329–33. PMID 3567071.
  3. ↑ Jackson N, Burt D, Crocker J, Boughton B (1987). "Skin mast cells in polycythaemia vera: relationship to the pathogenesis and treatment of pruritus". Br J Dermatol. 116 (1): 21–9. PMID 3814512.
  4. ↑ Fjellner B, Hägermark O (1979). "Pruritus in polycythemia vera: treatment with aspirin and possibility of platelet involvement". Acta Derm Venereol. 59 (6): 505–12. PMID 94209.
  5. ↑ Torgano G, Mandelli C, Massaro P, Abbiati C, Ponzetto A, Bertinieri G, Bogetto S, Terruzzi E, de Franchis R (2002). "Gastroduodenal lesions in polycythaemia vera: frequency and role of Helicobacter pylori". Br J Haematol. 117 (1): 198–202. PMID 11918555.
  6. ↑ van Genderen P, Michiels J (1997). "Erythromelalgia: a pathognomonic microvascular thrombotic complication in essential thrombocythemia and polycythemia vera". Semin Thromb Hemost. 23 (4): 357–63. PMID 9263352.
  7. ↑ Michiels J (1997). "Erythromelalgia and vascular complications in polycythemia vera". Semin Thromb Hemost. 23 (5): 441–54. PMID 9387203.
  8. ↑ Landolfi R, Ciabattoni G, Patrignani P, Castellana M, Pogliani E, Bizzi B, Patrono C (1992). "Increased thromboxane biosynthesis in patients with polycythemia vera: evidence for aspirin-suppressible platelet activation in vivo". Blood. 80 (8): 1965–71. PMID 1327286.

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