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Home  ›  If An Animal Has Been Diagnosed With Polycythemia, Which Of The Following Symptoms Typically Occurs?

If An Animal Has Been Diagnosed With Polycythemia, Which Of The Following Symptoms Typically Occurs?

Written By Thursday, May 12, 2022 Add Comment Edit

"erythremia" redirects here. For like-sounding medical terms, see arrythmia and erythema.

Medical condition

Polycythemia vera
Other names Polycythaemia vera (PV, PCV), erythremia, primary polycythemia, Vaquez affliction, Osler-Vaquez disease, polycythemia rubra vera[1]
Polycythemia vera, blood smear.jpg
Claret smear from a patient with polycythemia vera
Specialty Oncology

Polycythemia vera is an uncommon myeloproliferative neoplasm in which the bone marrow makes too many ruddy blood cells.[1] Information technology may also consequence in the overproduction of white blood cells and platelets.

Most of the health concerns associated with polycythemia vera are caused by the blood being thicker as a event of the increased red claret cells. Information technology is more common in the elderly and may exist symptomatic or asymptomatic. Common signs and symptoms include itching (pruritus), and astringent burning pain in the hands or anxiety that is normally accompanied past a cherry-red or bluish coloration of the pare. Patients with polycythemia vera are more likely to have gouty arthritis. Handling consists primarily of phlebotomy.

Signs and symptoms [edit]

Erythromelalgia is a rare symptom of PV, here present in a patient with longstanding polycythemia vera. Note reddish limbs and swelling.

People with polycythemia vera tin be asymptomatic.[2] A classic symptom of polycythemia vera is pruritus or itching, peculiarly after exposure to warm h2o (such equally when taking a bathroom),[3] which may be due to abnormal histamine release[4] [5] or prostaglandin production.[vi] Such itching is present in approximately 40% of patients with polycythemia vera.[7] Gouty arthritis may be present in upwards to 20% of patients.[7] Peptic ulcer disease is besides common in patients with polycythemia vera; nigh likely due to increased histamine from mast cells, simply may exist related to an increased susceptibility to infection with the ulcer-causing bacterium H. pylori.[8] Another possible mechanism for the development for peptic ulcer is increased histamine release and gastric hyperacidity related with polycythemia vera.[ citation needed ]

A classic symptom of polycythemia vera (and the related myeloproliferative illness essential thrombocythemia) is erythromelalgia.[nine] This is a burning hurting in the hands or feet, usually accompanied by a reddish or bluish coloration of the pare. Erythromelalgia is acquired by an increased platelet count or increased platelet "stickiness" (assemblage), resulting in the germination of tiny blood clots in the vessels of the extremity; information technology responds chop-chop to handling with aspirin.[x] [xi]

Patients with polycythemia vera are prone to the development of blood clots (thrombosis). A major thrombotic complication (e.g. heart attack, stroke, deep venous thrombosis, or Budd-Chiari syndrome) may sometimes exist the first symptom or indication that a person has polycythemia vera. Headaches, lack of concentration and fatigue are common symptoms that occur in patients with polycythemia vera as well.[ citation needed ]

Pathophysiology [edit]

Polycythemia vera (PCV), beingness a primary polycythemia, is caused by neoplastic proliferation and maturation of erythroid, megakaryocytic and granulocytic elements to produce what is referred to equally panmyelosis. In dissimilarity to secondary polycythemias, PCV is associated with a low serum level of the hormone erythropoietin (EPO). Instead, PCV cells frequently carry activating mutation in the tyrosine kinase (JAK2) gene, which acts in signaling pathways of the EPO-receptor, making those cells proliferate independent from EPO.[12]

Diagnosis [edit]

Physical exam findings are non-specific, but may include enlarged liver or spleen, plethora, or gouty nodules. The diagnosis is oft suspected on the basis of laboratory tests. Common findings include an elevated hemoglobin level and hematocrit, reflecting the increased number of ruby-red blood cells; the platelet count or white claret jail cell count may too be increased. The erythrocyte sedimentation rate (ESR) is decreased due to an increment in zeta potential. Because polycythemia vera results from an essential increase in erythrocyte production, patients have normal blood oxygenation and a low erythropoietin (EPO) level.[ citation needed ]

In primary polycythemia, in that location may exist 8 to ix million and occasionally 11 million erythrocytes per cubic millimeter of blood (a normal range for adults is 4-half-dozen), and the hematocrit may exist as high as seventy to 80%. In addition, the full blood volume sometimes increases to equally much as twice normal. The unabridged vascular arrangement can get markedly engorged with blood, and circulation times for blood throughout the body can increase upward to twice the normal value. The increased numbers of erythrocytes tin can crusade the viscosity of the blood to increment as much as five times normal. Capillaries can go plugged past the very pasty claret, and the period of blood through the vessels tends to be extremely sluggish.[ citation needed ]

As a result of the to a higher place, people with untreated polycythemia vera are at a adventure of various thrombotic events (deep venous thrombosis, pulmonary embolism), heart attack and stroke, and have a substantial gamble of Budd-Chiari syndrome (hepatic vein thrombosis),[xiii] or myelofibrosis. The status is considered chronic; no cure exists. Symptomatic treatment (see below) can normalize the claret count and most patients can live a normal life for years.[ citation needed ]

The disease appears more than mutual in Jews of European extraction than in most non-Jewish populations. Some familial forms of polycythemia vera are noted, only the mode of inheritance is non clear.[ commendation needed ]

A mutation in the JAK2 kinase (V617F) is strongly associated with polycythemia vera.[xiv] [15] JAK2 is a member of the Janus kinase family and makes the erythroid precursors hypersensitive to erythropoietin (EPO). This mutation may be helpful in making a diagnosis or equally a target for future therapy.

Following history and exam, the British Committee for Standards in Haematology (BCSH) recommend the following tests are performed:

  • full blood count/film (raised hematocrit; neutrophils, basophils, platelets raised in one-half of patients)
  • JAK2 mutation
  • serum ferritin
  • renal and liver function tests

If the JAK2 mutation is negative and in that location is no obvious secondary causes the BCSH suggest the following tests:

  • red cell mass
  • arterial oxygen saturation
  • abdominal ultrasound
  • serum erythropoietin level
  • bone marrow aspirate and trephine
  • cytogenetic analysis
  • erythroid burst-forming unit (BFU-E) civilisation

Other features that may exist seen in polycythemia vera include a low ESR and a raised leukocyte alkaline phosphatase.

The diagnostic criteria for polycythemia vera have recently been updated by the BCSH. This replaces the previous Polycythemia Vera Report Grouping criteria.

JAK2-positive polycythemia vera - diagnosis requires both criteria to be present:

Criteria Notes
A1 High erythrocyte volume fraction (EVF or hematocrit) (>0.52 in men, >0.48 in women) OR raised red jail cell mass (>25% to a higher place predicted)
A2 Mutation in JAK2

JAK2-negative polycythemia vera - diagnosis requires A1 + A2 + A3 + either another A or two B criteria:

Criteria Notes
A1 Raised cherry-red prison cell mass (>25% in a higher place predicted) OR hematocrit >0.60 in men, >0.56 in women
A2 Absence of mutation in JAK2
A3 No cause of secondary erythrocytosis
A4 Palpable splenomegaly
A5 Presence of an acquired genetic aberration (excluding BCR-ABL) in the hematopoietic cells
B1 Thrombocytosis (platelet count >450 * 109/fifty)
B2 Neutrophil leucocytosis (neutrophil count > x * 109/l in non-smokers; > 12.5*109/l in smokers)
B3 Radiological prove of splenomegaly
B4 Endogenous erythroid colonies or low serum erythropoietin

Treatment [edit]

Untreated, polycythemia vera tin be fatal.[xvi] [17] Data on the outcome of life-span of an individual with polycythemia vera is inconclusive due to the rarity of the disease, with some sources stating a normal life bridge, and others stating a x to xx year median survival rate.[eighteen] [19]

Frequent claret withdrawals (phlebotomy) is one form of treatment, which often may be combined with other therapies. The removal of claret from the trunk induces fe deficiency, thereby decreasing the hemoglobin / hematocrit level, and reducing the take chances of blood clots. Phlebotomy is typically performed to bring their hematocrit (red blood cell percentage) down below 45 for men or 42 for women.[twenty] It has been observed that phlebotomy also reduces cerebral damage.[21]

Medications are as well used which reduce the number of reddish blood cells. These include hydroxyurea and interferon therapy, among others.[22] The tendency of some practitioners to avoid chemotherapy if possible, especially in immature patients, is a result of enquiry indicating possible increased hazard of transformation to acute myelogenous leukemia (AML). While hydroxyurea is considered safer in this attribute, there is still some debate about its long-term safe.[23]

There are indications that the lung cancer drug erlotinib may be an boosted handling option for those with certain genetic markers.[24]

Ruxolitinib, a Janus kinase 2 (JAK2) inhibitor, is also used to treat polycythemia.[25]

Ropeginterferon alfa-2b (Besremi) was canonical for medical use in the European union in Feb 2019,[26] and in the United states of america in November 2021.[27] [28] Ropeginterferon alfa-2b is the first medication approved by the U.S. Food and Drug Assistants (FDA) to treat polycythemia vera that people can take regardless of their handling history, and the first interferon therapy specifically approved for polycythemia vera.[27] Interferon alfa-2b is besides used.[22]

Epidemiology [edit]

Polycythemia vera occurs in all historic period groups,[29] although the incidence increases with age. 1 study institute the median historic period at diagnosis to exist 60 years,[7] while a Mayo Clinic report in Olmsted Canton, Minnesota found that the highest incidence was in people anile 70–79 years.[30] The overall incidence in the Minnesota population was i.9 per 100,000 person-years, and the affliction was more than common in men than women.[30] A cluster effectually a toxic site was confirmed in northeast Pennsylvania in 2008.[31]

Notable deaths [edit]

  • Phyllis George (1949–2020), American sportscaster and former First Lady of Kentucky[32]
  • Ron Miles (1963–2022), American jazz trumpeter[33]
  • Nell Rankin (1924–2005), American mezzo-soprano[34]

References [edit]

  1. ^ a b "polycythemia vera." at Encyclopædia Britannica. 2010. Encyclopædia Britannica Online. 21 Sep. 2010
  2. ^ [Polycythemia vera EBSCO database] verified past URAC; accessed from Mountain Sinai Hospital, New York
  3. ^ Saini KS, Patnaik MM, Tefferi A (2010). "Polycythemia vera-associated pruritus and its management". Eur J Clin Invest. twoscore (nine): 828–34. doi:x.1111/j.1365-2362.2010.02334.x. PMID 20597963. S2CID 13638890.
  4. ^ Steinman H, Kobza-Black A, Lotti T, Brunetti L, Panconesi Due east, Greaves M (1987). "Polycythaemia rubra vera and water-induced pruritus: claret histamine levels and cutaneous fibrinolytic activity before and after h2o challenge". Br J Dermatol. 116 (iii): 329–33. doi:10.1111/j.1365-2133.1987.tb05846.x. PMID 3567071. S2CID 22068469.
  5. ^ Jackson Due north, 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. doi:10.1111/j.1365-2133.1987.tb05787.x. PMID 3814512. S2CID 38261640.
  6. ^ Fjellner B, Hägermark O (1979). "Pruritus in polycythemia vera: treatment with aspirin and possibility of platelet involvement". Acta Derm Venereol. 59 (6): 505–12. doi:10.2340/0001555559505512 (inactive 28 February 2022). PMID 94209. S2CID 6909368. {{cite journal}}: CS1 maint: DOI inactive equally of Feb 2022 (link)
  7. ^ a b c Berlin NI (1975). "Diagnosis and nomenclature of polycythemias". Semin Hematol. 12 (iv): 339–51. PMID 1198126.
  8. ^ Torgano M, Mandelli C, Massaro P, Abbiati C, Ponzetto A, Bertinieri Yard, Bogetto S, Terruzzi E, de Franchis R (2002). "Gastroduodenal lesions in polycythaemia vera: frequency and function of Helicobacter pylori". Br J Haematol. 117 (one): 198–202. doi:ten.1046/j.1365-2141.2002.03380.ten. PMID 11918555.
  9. ^ 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. doi:10.1055/south-2007-996109. PMID 9263352.
  10. ^ Michiels J (1997). "Erythromelalgia and vascular complications in polycythemia vera". Semin Thromb Hemost. 23 (5): 441–54. doi:10.1055/s-2007-996121. PMID 9387203.
  11. ^ Landolfi R, Ciabattoni Thousand, Patrignani P, Castellana Grand, Pogliani E, Bizzi B, Patrono C (1992). "Increased thromboxane biosynthesis in patients with polycythemia vera: bear witness for aspirin-suppressible platelet activation in vivo". Blood. eighty (8): 1965–71. doi:10.1182/blood.V80.8.1965.1965. PMID 1327286.
  12. ^ Kumar, et al.: Robbin'due south Basic Pathology, 8th edition, Saunders, 2007
  13. ^ Thurmes PJ, Steensma DP (July 2006). "Elevated serum erythropoietin levels in patients with Budd-Chiari syndrome secondary to polycythemia vera: clinical implications for the role of JAK2 mutation assay". Eur. J. Haematol. 77 (1): 57–60. doi:10.1111/j.1600-0609.2006.00667.ten. PMID 16827884. S2CID 37383942.
  14. ^ Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas North, Swanton S, Vassiliou GS, Demote AJ, Boyd EM, Curtin N, Scott MA, Erber WN, Greenish AR (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365 (9464): 1054–61. doi:ten.1016/S0140-6736(05)71142-9. PMID 15781101. S2CID 24419497.
  15. ^ Levine RL, Wadleigh Chiliad, Cools J, Ebert BL, Wernig 1000, Huntly BJ, Boggon TJ, Wlodarska I, Clark JJ, Moore S, Adelsperger J, Koo S, Lee JC, Gabriel South, Mercher T, D'Andrea A, Frohling S, Dohner K, Marynen P, Vandenberghe P, Mesa RA, Tefferi A, Griffin JD, Eck MJ, Sellers WR, Meyerson M, Golub TR, Lee SJ, Gilliland DG (2005). "Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell. 7 (four): 387–97. doi:10.1016/j.ccr.2005.03.023. PMID 15837627.
  16. ^ Mayo Clinic staff. "Polycythemia vera - MayoClinic.com". Polycythemia vera: Definition. Mayo Clinic. Retrieved 2011-09-03 .
  17. ^ "What Is Polycythemia Vera?". National Centre, Lung and Blood Institute. Retrieved 2011-09-03 .
  18. ^ "Polycythemia Vera Follow-upwards". Retrieved 2011-09-03 .
  19. ^ "Highlights in Polycythemia Vera From the 2016 EHA Congres". Retrieved 2016-ten-01 .
  20. ^ Streiff MB, Smith B, Spivak JL (2002). "The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Written report Group: a survey of American Society of Hematology members' practice patterns". Blood. 99 (4): 1144–ix. doi:10.1182/blood.V99.iv.1144. PMID 11830459.
  21. ^ Di Pollina L, Mulligan R, Juillerat Van der Linden A, Michel JP, Gilt G (2000). "Cognitive impairment in polycythemia vera: partial reversibility upon lowering of the hematocrit". Eur. Neurol. 44 (1): 57–nine. doi:x.1159/000008194. PMID 10894997. S2CID 40928145.
  22. ^ a b "Polycythemia vera - Diagnosis and treatment - Mayo Clinic". www.mayoclinic.org . Retrieved 2022-03-xi .
  23. ^ Björkholm, M; Derolf, AR; Hultcrantz, M; et al. (ten June 2011). "Treatment-related adventure factors for transformation to astute myeloid leukemia and myelodysplastic syndromes in myeloproliferative neoplasms". Journal of Clinical Oncology. 29 (17): 2410–5. doi:x.1200/JCO.2011.34.7542. PMC3107755. PMID 21537037.
  24. ^ Li Z, Xu M, Xing S, Ho West, Ishii T, Li Q, Fu 10, Zhao Z (2007). "Erlotinib Effectively Inhibits JAK2V617F Activity and Polycythemia Vera Jail cell Growth". J Biol Chem. 282 (6): 3428–32. doi:10.1074/jbc.C600277200. PMC2096634. PMID 17178722.
  25. ^ Tefferi, A; Vannucchi, AM; Barbui, T (10 January 2018). "Polycythemia vera treatment algorithm 2018". Blood Cancer Journal. 8 (1): iii. doi:10.1038/s41408-017-0042-vii. PMC5802495. PMID 29321547.
  26. ^ "Besremi EPAR". European Medicines Bureau (EMA) . Retrieved 14 Nov 2021.
  27. ^ a b "FDA Approves Treatment for Rare Claret Disease". U.S. Food and Drug Administration (FDA) (Printing release). 12 November 2021. Retrieved 12 November 2021. Public Domain This article incorporates text from this source, which is in the public domain .
  28. ^ "U.South. FDA Approves Besremi (ropeginterferon alfa-2b-njft) every bit the Only Interferon for Adults With Polycythemia Vera" (Printing release). PharmaEssentia. 12 November 2021. Retrieved 14 November 2021 – via Business Wire.
  29. ^ Passamonti F, Malabarba 50, Orlandi Eastward, Baratè C, Canevari A, Brusamolino Eastward, Bonfichi Grand, Arcaini L, Caberlon S, Pascutto C, Lazzarino M (2003). "Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia". Haematologica. 88 (1): xiii–8. PMID 12551821.
  30. ^ a b Anía B, Suman V, Sobell J, Codd M, Silverstein Thou, Melton L (1994). "Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989". Am J Hematol. 47 (2): 89–93. doi:10.1002/ajh.2830470205. PMID 8092146. S2CID 31536624.
  31. ^ MICHAEL RUBINKAM (2008). "Cancer cluster confirmed in northeast Pennsylvania". Associated Press. Archived from the original on September ii, 2008.
  32. ^ Yetter, Deborah (May 16, 2020). "Phyllis George, former Kentucky kickoff lady and Miss America, dies at 70". The Courier-Journal . Retrieved May xvi, 2020.
  33. ^ Harrington, Jim (March nine, 2022). "'Gifted artist' Ron Miles dies of a rare claret disorder at 58". The Mercury News . Retrieved March ten, 2022.
  34. ^ Allan Kozinn (Jan 19, 2005). "Nell Rankin Is Dead at 81; Mezzo-Soprano With Met". The New York Times.

External links [edit]

  • Polycythemia Vera National Eye, Lung, and Blood Establish
  • 11-141d. at Merck Manual of Diagnosis and Therapy Professional Edition

Source: https://en.wikipedia.org/wiki/Polycythemia_vera

Posted by: browntheird.blogspot.com

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