Difference between revisions of "Polycythemia vera natural history, complications, and prognosis"

Jump to: navigation, search
(Complications)
 
Line 1: Line 1:
 
__NOTOC__
 
__NOTOC__
 
{{Polycythemia vera}}
 
{{Polycythemia vera}}
{{CMG}}{{AE}}{{MJK}} {{shyam}}
+
{{CMG}}{{AE}} {{IO}} {{MJK}} {{shyam}}
 
==Overview==
 
==Overview==
 
The natural history of polycythemia vera begins with symptoms such as [[headache]], [[fatigue]], and [[dyspnea]]. Common complications of polycythemia vera include [[thrombosis]] (such as [[deep vein thrombosis]], [[pulmonary embolism]], [[myocardial infarction]], and [[stroke]]), [[bleeding]], and [[splenomegaly]]. Prognosis is generally good with treatment, and the median [[Survival rates|survival]] for patients with polycythemia vera is around 10.9 to 27.8 years in the absence of complications. However, there is a variable risk for progression to [[myelofibrosis]] and acute myeloid leukemia, and these are the most devastating complications of the disease. [[Myelofibrosis]] and [[acute myeloid leukemia]] are part of the natural history of the disease.
 
The natural history of polycythemia vera begins with symptoms such as [[headache]], [[fatigue]], and [[dyspnea]]. Common complications of polycythemia vera include [[thrombosis]] (such as [[deep vein thrombosis]], [[pulmonary embolism]], [[myocardial infarction]], and [[stroke]]), [[bleeding]], and [[splenomegaly]]. Prognosis is generally good with treatment, and the median [[Survival rates|survival]] for patients with polycythemia vera is around 10.9 to 27.8 years in the absence of complications. However, there is a variable risk for progression to [[myelofibrosis]] and acute myeloid leukemia, and these are the most devastating complications of the disease. [[Myelofibrosis]] and [[acute myeloid leukemia]] are part of the natural history of the disease.

Latest revision as of 03:05, 14 September 2019

Polycythemia vera Microchapters

Home

Patient Information

Overview

Historical perspective

Classification

Pathophysiology

Causes

Differentiating Polycythemia vera from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural history, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Polycythemia vera natural history, complications, and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Polycythemia vera natural history, complications, and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Polycythemia vera natural history, complications, and prognosis

CDC on Polycythemia vera natural history, complications, and prognosis

Polycythemia vera natural history, complications, and prognosis in the news

Blogs on Polycythemia vera natural history, complications, and prognosis

Directions to Hospitals Treating Polycythemia vera

Risk calculators and risk factors for Polycythemia vera natural history, complications, and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Ifeoma Odukwe M.D. [2] Mohamad Alkateb, MBBCh [3] Shyam Patel [4]

Overview

The natural history of polycythemia vera begins with symptoms such as headache, fatigue, and dyspnea. Common complications of polycythemia vera include thrombosis (such as deep vein thrombosis, pulmonary embolism, myocardial infarction, and stroke), bleeding, 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 in the absence of complications. However, there is a variable risk for progression to myelofibrosis and acute myeloid leukemia, and these are the most devastating complications of the disease. Myelofibrosis and acute myeloid leukemia are part of the natural history of the disease.

Natural History

  • The symptoms of polycythemia vera usually develop in the sixth decade of life and start with symptoms such as headache and fatigue.
  • The cumulative risk of developing myelofibrosis is 4.9 to 6.0% at 10 years, 6-14% at 15 years, and 26% at 20 years from the initial diagnosis of polycythemia vera. This is in comparison to the risk of post-essential thrombocythemia myelofibrosis, for which the risk is 0.8% to 4.9% at 10 years and 4-11% at 15 years.[1][2]
  • The cumulative risk of developing acute myeloid leukemia is 2.3-14% at 10 years, 5.5-18% at 15 years, and greater than 10% at 20 years from the initial diagnosis of polycythemia vera. This is in comparison to the risk of post-ET acute myeloid leukemia, for which the risk is 0.7-3% at 10 years and 2.1-5.3% at 15 years.[1][2]

Complications

Common complications of polycythemia vera include:[1][3][4][5][6][7]

Prognosis

Life expectancy

  • After transformation of polycythemia vera to myelofibrosis, the median survival is approximately 5.7 years.[9]
  • The median survival for patients with acute myeloid leukemia that has progressed from polycythemia vera is 5 months.[9]
  • High-risk patients with polycythemia vera have an average survival of 10.9 years. Low-risk patients have an average survival of 27.8 years.[9]
  • There is a 4.2-fold increase in the risk of death in the presence of any of the following risk factors[2]:
  • The 5-year survival rate is 17.3% for patients who develop post-PV myelofibrosis and have hemoglobin < 10 g/dl and age above 65 years.[2]
  • In elderly patients compared to younger patients, leukemic transformation contributes to more deaths, suggesting that age of onset of leukemia is an important prognostic risk factor for life expectancy.[2]

References

  1. 1.0 1.1 1.2 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.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Cerquozzi S, Tefferi A (2015). "Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors". Blood Cancer J. 5: e366. doi:10.1038/bcj.2015.95. PMC 4670948. PMID 26565403.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. "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.
  8. 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.
  9. 9.0 9.1 9.2 9.3 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.



Linked-in.jpg