Polycythemia vera pathophysiology
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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
Polycythemia vera arises from hematopoietic stem cells, which give rise to erythrocytes cells that are normally involved in delivering oxygen to the body tissue.[1]
Pathophysiology
In primary polycythemia, there might be 8 to 9 million erythrocytes per cubic millimeter of blood, and the hematocrit may be as high as 70 to 80%. In addition, the total blood volume sometimes increases to as much as twice the normal values. The entire vascular system can become markedly engorged with blood, and the blood circulation time throughout the body can increase up to twice the normal value. The increased numbers of erythrocytes causes a five time increase in blood viscosity. Capillaries can become plugged by the very viscous blood which results in an extremely sluggish flow of blood.[2][3]
Genetics
Gene involved in the pathogenesis of polycythemia vera include JAK2 kinase (V617F).[4][5]
References
- ↑ Jamieson CH, Gotlib J, Durocher JA, Chao MP, Mariappan MR, Lay M; et al. (2006). "The JAK2 V617F mutation occurs in hematopoietic stem cells in polycythemia vera and predisposes toward erythroid differentiation". Proc Natl Acad Sci U S A. 103 (16): 6224–9. doi:10.1073/pnas.0601462103. PMC 1434515. PMID 16603627.
- ↑ 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 analysis". Eur. J. Haematol. 77 (1): 57–60. doi:10.1111/j.1600-0609.2006.00667.x. PMID 16827884.
- ↑ National Cancer Institute. Polycythemia vera.https://en.wikipedia.org/wiki/Polycythemia_vera
- ↑ Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, Vassiliou GS, Bench AJ, Boyd EM, Curtin N, Scott MA, Erber WN, Green AR (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365 (9464): 1054–61. PMID 15781101.
- ↑ Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, Boggon TJ, Wlodarska I, Clark JJ, Moore S, Adelsperger J, Koo S, Lee JC, Gabriel S, 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 (4): 387–97. PMID 15837627.