Polycythemia vera natural history, complications, and prognosis

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

Overview

If left untreated, patients with polycythemia vera may progress to develop headache, fatigue, and dyspnea. Common complications of polycythemia vera include bleeding, thrombosis, tinnitus , and splenomegaly. Prognosis is generally good with treatment, and the median survival for patients with polycythemia vera is around 10.9 to 27.8 years.[1]

Natural History

The symptoms of polycythemia vera usually develop in the sixth decade of life and start with symptoms such as headache and fatigue.[2] If left untreated, the natural history of polycythemia vera will result in post-polycythemia vera (post-PV) myelofibrosis and acute myeloid leukemla (AML).[3] The median time to development of myelofibrosis is 8-20 years. The cumulative risk of developing myelofibrosis is 6% at 10 years, 14% at 15 years, and 26% at 20 years from the initial diagnosis of polycythemia vera.[3]

Complications

Polycythemia vera may lead to the following complications:[2][4][5][6][7][8]

  • Epistaxis: Bleeding can occur in the anterior or posterior circulation of the nasal cavity.
  • Gingival gums: Bleeding gums is typically seen after brushing teeth.
  • Menorrhagia: Pelvis bleeding commonly occurs in pre-menopausal females.
  • Metrorrhagia: Irregular pelvic bleeding can occur in pre-menopausal females.
  • Petechiae: Pinpoint hemorrhages can occur in the skin.
  • Myelofibrosis: This is a condition in which the bone marrow becomes replaced by collagen and reticulin fibers. When myelofibrosis occurs in the setting of polycythemia vera, it is referred to as post-polycythemia vera (post-PV) myelofibrosis. Myelofibrosis carries an overall poor prognosis given that collagen fibers preclude normal hematopoiesis, resulting in infections, bleeding, and fatigue.
  • Acute myeloid leukemia: This is a malignancy of the hematopoietic stem cell (specifically myeloid precursors). It is characterized by clonal proliferation and resultant cytopenias and ineffective hematopoiesis. Patients typically die as a result of infections and/or bleeding.

Prognosis

The prognosis of polycythemia vera is good with treatment. Without treatment, polycythemia vera will result in death.[1] Once myelofibrosis or acute myeloid leukemia ensues, the prognosis is very poor.

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References

  1. 1.0 1.1 Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F; et al. (2013). "Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study". Leukemia. 27 (9): 1874–81. doi:10.1038/leu.2013.163. PMC 3768558. PMID 23739289.
  2. 2.0 2.1 Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab
  3. 3.0 3.1 Vannucchi AM (2017). "From leeches to personalized medicine: evolving concepts in the management of polycythemia vera". Haematologica. 102 (1): 18–29. doi:10.3324/haematol.2015.129155. PMC 5210229. PMID 27884974.
  4. 4.0 4.1 Zoraster RM, Rison RA (2013). "Acute embolic cerebral ischemia as an initial presentation of polycythemia vera: a case report". J Med Case Rep. 7: 131. doi:10.1186/1752-1947-7-131. PMC 3668271. PMID 23683307.
  5. Buzas C, Sparchez Z, Cucuianu A, Manole S, Lupescu I, Acalovschi M (2009). "Budd-Chiari syndrome secondary to polycythemia vera. A case report". J Gastrointestin Liver Dis. 18 (3): 363–6. PMID 19795034.
  6. Biagioni E, Pedrazzi P, Marietta M, Di Benedetto F, Villa E, Luppi M; et al. (2013). "Successful liver transplantation in a patient with splanchnic vein thrombosis and pulmonary embolism due to polycythemia vera with Jak2v617f mutation and heparin-induced thrombocytopenia". J Thromb Thrombolysis. 36 (3): 352–4. doi:10.1007/s11239-012-0832-5. PMID 23277116.
  7. Reikvam H, Tiu RV (2012). "Venous thromboembolism in patients with essential thrombocythemia and polycythemia vera". Leukemia. 26 (4): 563–71. doi:10.1038/leu.2011.314. PMID 22076463.
  8. "Erratum: Borderud SP, Li Y, Burkhalter JE, Sheffer CE and Ostroff JS. Electronic cigarette use among patients with cancer: Characteristics of electronic cigarette users and their smoking cessation outcomes. Cancer. doi: 10.1002/ cncr.28811". Cancer. 121 (5): 800. 2015. PMID 25855820.

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