Abstract
Polycythemia vera (PV) is a clonal myeloproliferative disease characterized by erythrocytosis. This is pathologically characterized by the simultaneous proliferation of erythroid, granulocyte, and megakaryocytes in bone marrow. The number and the capacity of erythrocyte are significantly increased in clinical characteristics, accompanied by neutrophils and thrombocytosis, and a series of symptoms and signs caused by multiple blood and hyperviscosity. PV is often accompanied by splenomegaly. The onset of PV is insidious, in which the progress is slow and various transformations can occur in the late stage. Approximately half of the patients develop hypertension, usually with elevated systolic blood pressure.
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References
Anía BJ, Suman VJ, Sobell JL, et al. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989. Am J Hematol. 1994;47:89.
Tefferi A, Spivak JL. Polycythemia vera: scientific advances and current practice. Semin Hematol. 2005;42:206.
Tefferi A, Rumi E, Finazzi G, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27:1874.
Kralovics R, Passamonti F, Buser AS, Teo SS, Tiedt R, Passweg JR, et al. A gain-of-function mutation of JAK2 in myelopro- liferative disorders. N Engl J Med. 2005;352:1779–90.
Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocytemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005;7:387–97.
Scott LM, Tong W, Levine RL, Scott MA, Beer PA, Stratton MR, et al. JAK2 eon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N Engl J Med. 2007;356:459–68.
Wang YL, Vandris K, Jones A, et al. JAK2 mutations are present in all cases of polycythemia vera. Leukemia. 2008;22:1289.
Pardanani A, Lasho TL, Finke C, et al. Prevalence and clinicopathologic correlates of JAK2 exon 12 mutations in JAK2V617F-negative polycythemia vera. Leukemia. 2007;21:1960.
Lasho TL, Pardanani A, Tefferi A. LNK mutations in JAK2 mutation-negative erythrocytosis. N Engl J Med. 2010;363:1189.
Scott LM. The JAK2 exon 12 mutations: a comprehensive review. Am J Hematol. 2011;86:668.
Rumi E, Pietra D, Ferretti V, et al. JAK2 or CALR mutation status defines subtypes of essential thrombocythemia with substantially different clinical course and outcomes. Blood. 2013;123(10):1544–51.
Siegel FP, Tauscher J, Petrides PE. Aquagenic pruritus in polycythemia vera: characteristics and influence on quality of life in 441 patients. Am J Hematol. 2013;88:665.
Michiels JJ. Erythromelalgia and vascular complications in polycythemia vera. Semin Thromb Hemost. 1997;23:441.
Chait Y, Condat B, Cazals-Hatem D, et al. Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis. Br J Haematol. 2005;129:553.
Ruggeri M, Rodeghiero F, Tosetto A, et al. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: a retrospective survey. Blood. 2008;111:666.
Kralovics R, Buser AS, Teo SS, et al. Comparison of molecular markers in a cohort of patients with chronic myeloproliferative disorders. Blood. 2003;102:1869.
Andrieux JL, Demory JL. Karyotype and molecular cytogenetic studies in polycythemia vera. Curr Hematol Rep. 2005;4:224.
Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391–405.
McMullin MF, Harrison CN, Ali S, et al. A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology guideline. Br J Haematol. 2019;184(2):176–91.
Marchioli R, Finazzi G, Specchia G, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013;368(1):22–33.
Chinese expert consensus on diagnosis and treatment of polycythemia vera (2016 edition). Chinese J Hematol. 2016;37(4):852–857.
Lver RT, Kiladjian JJ, Hasselbalch HC. Interferon and the treatment of polycythemia vera, essential thrombocythemia and myelofibrosis. Expert Rev Hematol. 2013;6(1):49–58.
Büyükaşik Y, Alİ R, Ar C, et al. Polycythemia vera: diagnosis, clinical course, and current management. Turk J Med Sci. 2018;48(4):698–710.
Vannucchi AM, Kiladjian JJ, Griesshammer M, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015;372(5):426–35.
Hao-Zhu C, Lin G-W. Practical internal medicine. 13th ed. Beijing: People’s Health Publishing House.
Badireddy M. Chronic Anemia[J]. null, 2019
Moodalbail DG, Falkner B, Keith SW, et al. Ambulatory hypertension in a pediatric cohort of sickle cell disease. J Am Soc Hypertens. 2018;12(7):542–50.
Mikhail A, Brown C, Williams JA, et al. Renal association clinical practice guideline on Anaemia of Chronic Kidney Disease. BMC Nephrol. 2017;18(1):345.
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Wang, L., Shi, A., Maimaiti, N. (2020). Hematological System Diseases. In: Li, N. (eds) Secondary Hypertension. Springer, Singapore. https://doi.org/10.1007/978-981-15-0591-1_16
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