Polycythemia vera natural history, complications, and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Polycythemia vera}} | {{Polycythemia vera}} | ||
{{CMG}}{{AE}}{{MJK}} | {{CMG}}{{AE}}{{MJK}} {{shyam}} | ||
==Overview== | ==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.<ref name="pmid23739289">{{cite journal| author=Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F et al.| title=Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. | journal=Leukemia | year= 2013 | volume= 27 | issue= 9 | pages= 1874-81 | pmid=23739289 | doi=10.1038/leu.2013.163 | pmc=PMC3768558 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23739289 }} </ref> | 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.<ref name="pmid23739289">{{cite journal| author=Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F et al.| title=Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. | journal=Leukemia | year= 2013 | volume= 27 | issue= 9 | pages= 1874-81 | pmid=23739289 | doi=10.1038/leu.2013.163 | pmc=PMC3768558 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23739289 }} </ref> | ||
==Natural History== | ==Natural History== | ||
The symptoms of polycythemia vera usually develop in the sixth decade of life | The symptoms of polycythemia vera usually develop in the sixth decade of life and start with symptoms such as [[headache]] and [[fatigue]].<ref name="cancer.ca">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab</ref> If left untreated, the natural history of polycythemia vera will result in post-polycythemia vera (post-PV) myelofibrosis and acute myeloid leukemla (AML).<ref name="pmid27884974">{{cite journal| author=Vannucchi AM| title=From leeches to personalized medicine: evolving concepts in the management of polycythemia vera. | journal=Haematologica | year= 2017 | volume= 102 | issue= 1 | pages= 18-29 | pmid=27884974 | doi=10.3324/haematol.2015.129155 | pmc=5210229 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27884974 }} </ref> | ||
==Complications== | ==Complications== | ||
Polycythemia vera may lead to the following complications:<ref name="cancer.ca">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab</ref><ref name="pmid23683307">{{cite journal| author=Zoraster RM, Rison RA| title=Acute embolic cerebral ischemia as an initial presentation of polycythemia vera: a case report. | journal=J Med Case Rep | year= 2013 | volume= 7 | issue= | pages= 131 | pmid=23683307 | doi=10.1186/1752-1947-7-131 | pmc=PMC3668271 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23683307 }} </ref><ref name="pmid19795034">{{cite journal| author=Buzas C, Sparchez Z, Cucuianu A, Manole S, Lupescu I, Acalovschi M| title=Budd-Chiari syndrome secondary to polycythemia vera. A case report. | journal=J Gastrointestin Liver Dis | year= 2009 | volume= 18 | issue= 3 | pages= 363-6 | pmid=19795034 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19795034 }} </ref><ref name="pmid23277116">{{cite journal| author=Biagioni E, Pedrazzi P, Marietta M, Di Benedetto F, Villa E, Luppi M et al.| title=Successful liver transplantation in a patient with splanchnic vein thrombosis and pulmonary embolism due to polycythemia vera with Jak2v617f mutation and heparin-induced thrombocytopenia. | journal=J Thromb Thrombolysis | year= 2013 | volume= 36 | issue= 3 | pages= 352-4 | pmid=23277116 | doi=10.1007/s11239-012-0832-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23277116 }} </ref><ref name="pmid22076463">{{cite journal| author=Reikvam H, Tiu RV| title=Venous thromboembolism in patients with essential thrombocythemia and polycythemia vera. | journal=Leukemia | year= 2012 | volume= 26 | issue= 4 | pages= 563-71 | pmid=22076463 | doi=10.1038/leu.2011.314 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22076463 }} </ref><ref name="pmid25855820">{{cite journal| author=| title=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. | journal=Cancer | year= 2015 | volume= 121 | issue= 5 | pages= 800 | pmid=25855820 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25855820 }} </ref> | Polycythemia vera may lead to the following complications:<ref name="cancer.ca">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/polycythemia-vera/?region=ab</ref><ref name="pmid23683307">{{cite journal| author=Zoraster RM, Rison RA| title=Acute embolic cerebral ischemia as an initial presentation of polycythemia vera: a case report. | journal=J Med Case Rep | year= 2013 | volume= 7 | issue= | pages= 131 | pmid=23683307 | doi=10.1186/1752-1947-7-131 | pmc=PMC3668271 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23683307 }} </ref><ref name="pmid19795034">{{cite journal| author=Buzas C, Sparchez Z, Cucuianu A, Manole S, Lupescu I, Acalovschi M| title=Budd-Chiari syndrome secondary to polycythemia vera. A case report. | journal=J Gastrointestin Liver Dis | year= 2009 | volume= 18 | issue= 3 | pages= 363-6 | pmid=19795034 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19795034 }} </ref><ref name="pmid23277116">{{cite journal| author=Biagioni E, Pedrazzi P, Marietta M, Di Benedetto F, Villa E, Luppi M et al.| title=Successful liver transplantation in a patient with splanchnic vein thrombosis and pulmonary embolism due to polycythemia vera with Jak2v617f mutation and heparin-induced thrombocytopenia. | journal=J Thromb Thrombolysis | year= 2013 | volume= 36 | issue= 3 | pages= 352-4 | pmid=23277116 | doi=10.1007/s11239-012-0832-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23277116 }} </ref><ref name="pmid22076463">{{cite journal| author=Reikvam H, Tiu RV| title=Venous thromboembolism in patients with essential thrombocythemia and polycythemia vera. | journal=Leukemia | year= 2012 | volume= 26 | issue= 4 | pages= 563-71 | pmid=22076463 | doi=10.1038/leu.2011.314 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22076463 }} </ref><ref name="pmid25855820">{{cite journal| author=| title=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. | journal=Cancer | year= 2015 | volume= 121 | issue= 5 | pages= 800 | pmid=25855820 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25855820 }} </ref> | ||
*[[Thrombosis]] | *[[Thrombosis]] | ||
:*[[Deep venous thrombosis]] | :*[[Deep venous thrombosis]]: Clots can develop in the deep veins of the lower extremities, such as the [[popliteal vein]] or [[femoral vein]]. | ||
:*[[Pulmonary embolism]] | :*[[Pulmonary embolism]]: Clots can develop in the segmental or subsegmental arteries of the pulmonary vasculature. | ||
:*[[Myocardial infarction]] | :*[[Myocardial infarction]]: Clots can develop within the coronary arteries, and this is usually exacerbated by underlying cardiac risk factors. | ||
:*[[Stroke]] | :*[[Stroke]]: Clots can develop in the anterior or posterior circulation in the brain. | ||
:*[[Budd-Chiari syndrome]] | :*[[Budd-Chiari syndrome]]: Clots can develop in the hepatic vein. | ||
:*Splanchnic vein thrombosis | :*[[Splanchnic vein thrombosis]]: Clots can develop in mesenteric vasculature like the [[splenic vein]], [[superior mesenteric vein]], [[inferior mesenteric vein]], or [[portal vein]]. | ||
*[[Bleeding]] | *[[Bleeding]] | ||
:*[[Epistaxis]] | :*[[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]] | :*[[Menorrhagia]]: Pelvis bleeding commonly occurs in pre-menopausal females. | ||
:*[[Metrorrhagia]] | :*[[Metrorrhagia]]: Irregular pelvic bleeding can occur in pre-menopausal females. | ||
:*[[Petechiae]] | :*[[Petechiae]]: Pinpoint hemorrhages can occur in the skin. | ||
*[[ | *[[Splenomegaly]]: [[Extramedullary hematopoiesis]] occurs when the bone marrow is unable to sustain normal cell production. | ||
*[[Gout]] | *[[Gout]] | ||
*[[Peptic ulcer]] | *[[Peptic ulcer]] | ||
Revision as of 01:13, 21 January 2018
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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]
Complications
Polycythemia vera may lead to the following complications:[2][4][5][6][7][8]
- 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
Prognosis
The prognosis of polycythemia vera is good with treatment. Without treatment, polycythemia vera will result in death.[1]
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
- ↑ 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.