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]
- The risk of developing acute myeloid leukemia is 2% at 10 years, 5% at 15 years, and greater than 10% 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]
- Thrombosis: Clot formation occurs in 15% of patients with polycythemia vera.
- Deep venous thrombosis: Clots can develop in the deep veins of the lower extremities, such as the popliteal vein or femoral vein.
- Pulmonary embolism: Clots can develop in the segmental or subsegmental arteries of the pulmonary vasculature.
- Myocardial infarction: Clots can develop within the coronary arteries, and this is usually exacerbated by underlying cardiac risk factors.
- Stroke: Clots can develop in the anterior or posterior circulation in the brain.
- Budd-Chiari syndrome: Clots can develop in the hepatic vein.
- Splanchnic vein thrombosis: Clots can develop in mesenteric vasculature like the splenic vein, superior mesenteric vein, inferior mesenteric vein, or portal vein.
- 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.
- Splenomegaly: Extramedullary hematopoiesis occurs when the bone marrow is unable to sustain normal cell production.
- Gout
- Peptic ulcer
- 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.
- Hearing impairment
- Visual impairment
- Paresthesia
- Headache
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. The median survival for patients with acute myeloid leukemia that has progressed from polycythemia vera is 5 months. High-risk patients with polycythemia vera have an average survival of 10.9 years. Low-risk patients have an average survival of 29 months.[9] Prognosis is adversely affected by older age, presence of leukocytosis, and history of venous thromboembolic events.[9]
Gallery
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![Magnetic resonance imaging of the brain. Diffusion-weighted magnetic resonance image of the brain demonstrating numerous small foci of restricted diffusion scattered within the left frontoparietal cortex, subcortical white matter, and centrum semiovale. These foci are consistent with an acute embolic ischemic infarction shower within the left middle cerebral artery distribution.[4]](/images/b/b9/MRI-PV.jpeg)
Magnetic resonance imaging of the brain. Diffusion-weighted magnetic resonance image of the brain demonstrating numerous small foci of restricted diffusion scattered within the left frontoparietal cortex, subcortical white matter, and centrum semiovale. These foci are consistent with an acute embolic ischemic infarction shower within the left middle cerebral artery distribution.[4]
![Magnetic resonance imaging of the brain. Diffusion-weighted magnetic resonance image of the brain demonstrating numerous small foci of restricted diffusion scattered within the left frontoparietal cortex, subcortical white matter, and centrum semiovale. These foci are consistent with an acute embolic ischemic infarction shower within the left middle cerebral artery distribution.[4]](/index.php/File:MRI-PV.jpeg)
References
- ↑ 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.
- ↑ 2.0 2.1 Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab
- ↑ 3.0 3.1 3.2 3.3 3.4 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.
- ↑ 4.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ "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.
- ↑ 9.0 9.1 Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK; et al. (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.