Polycythemia vera history and symptoms: Difference between revisions

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{{CMG}}
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{{Polycythemia vera}}
{{Polycythemia vera}}
{{CMG}} {{AE}} {{IO}} {{MJK}}; {{shyam}}
==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]] especially after exposure to warm water, loss of concentration and [[night sweats]] and neurological symptoms.  
==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>
===History===
Patients with polycythemia vera may have a positive history of:<ref name="Tefferi2002">{{cite journal|last1=Tefferi|first1=A.|title=Selective serotonin reuptake inhibitors are effective in the treatment of polycythemia vera-associated pruritus|journal=Blood|volume=99|issue=7|year=2002|pages=2627–2627|issn=00064971|doi=10.1182/blood.V99.7.2627}}</ref>
*[[Pruritus]]
*[[Fatigue]]
*[[Headache]]
*[[Early satiety]]


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>
===Common Symptoms===
Common symptoms of polycythemia vera include:<ref name="Tefferi2002">{{cite journal|last1=Tefferi|first1=A.|title=Selective serotonin reuptake inhibitors are effective in the treatment of polycythemia vera-associated pruritus|journal=Blood|volume=99|issue=7|year=2002|pages=2627–2627|issn=00064971|doi=10.1182/blood.V99.7.2627}}</ref><ref name="pmid19468728">{{cite journal |vauthors=Cario H, McMullin MF, Pahl HL |title=Clinical and hematological presentation of children and adolescents with polycythemia vera |journal=Ann. Hematol. |volume=88 |issue=8 |pages=713–9 |date=August 2009 |pmid=19468728 |pmc=4135082 |doi=10.1007/s00277-009-0758-y |url=}}</ref><ref name="pmid20597963">{{cite journal |vauthors=Saini KS, Patnaik MM, Tefferi A |title=Polycythemia vera-associated pruritus and its management |journal=Eur. J. Clin. Invest. |volume=40 |issue=9 |pages=828–34 |date=September 2010 |pmid=20597963 |doi=10.1111/j.1365-2362.2010.02334.x |url=}}</ref>
*[[Fatigue]]
*[[Headache]]
*[[Pruritus]]
**Especially after exposure to warm water
**May be related to [[serotonin]] release by [[Platelet|platelets]] as well as elevated [[histamine]] levels
*Abdominal pain
*Loss of concentration
*[[Night sweats]]
*Sexual problems
*[[Insomnia]]
*Decreased mood
*[[Numbness]]
*[[Early satiety]]
*[[Dizziness]]
*[[Cough]]
*[[Erythromelalgia]]
*[[Weakness]]


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.
===Less Common Symptoms===
Less common symptoms of polycythemia vera include:<ref name="pmid26324368">{{cite journal |vauthors=Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R |title=Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F |journal=J. Clin. Oncol. |volume=33 |issue=33 |pages=3953–60 |date=November 2015 |pmid=26324368 |pmc=4979103 |doi=10.1200/JCO.2015.61.6474 |url=}}</ref>
*[[Fever]]
*Weight loss
*Sweating
*Visual disturbances
*[[Parasthesias|Paresthesia]]
*[[Dyspnea]]
*Bone pains


==References==
==References==
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Latest revision as of 03:06, 14 September 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2] Mohamad Alkateb, MBBCh [3]; Shyam Patel [4]

Overview

People with polycythemia vera usually asymptomatic. Symptoms of polycythemia vera include headache, fatigue, and pruritis especially after exposure to warm water, loss of concentration and night sweats and neurological symptoms.

History and Symptoms

History

Patients with polycythemia vera may have a positive history of:[1]

Common Symptoms

Common symptoms of polycythemia vera include:[1][2][3]

Less Common Symptoms

Less common symptoms of polycythemia vera include:[4]

References

  1. ↑ 1.0 1.1 Tefferi, A. (2002). "Selective serotonin reuptake inhibitors are effective in the treatment of polycythemia vera-associated pruritus". Blood. 99 (7): 2627–2627. doi:10.1182/blood.V99.7.2627. ISSN 0006-4971.
  2. ↑ Cario H, McMullin MF, Pahl HL (August 2009). "Clinical and hematological presentation of children and adolescents with polycythemia vera". Ann. Hematol. 88 (8): 713–9. doi:10.1007/s00277-009-0758-y. PMC 4135082. PMID 19468728.
  3. ↑ Saini KS, Patnaik MM, Tefferi A (September 2010). "Polycythemia vera-associated pruritus and its management". Eur. J. Clin. Invest. 40 (9): 828–34. doi:10.1111/j.1365-2362.2010.02334.x. PMID 20597963.
  4. ↑ Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R (November 2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F". J. Clin. Oncol. 33 (33): 3953–60. doi:10.1200/JCO.2015.61.6474. PMC 4979103. PMID 26324368.

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