Polycythemia vera natural history, complications, and prognosis
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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.
- 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.
- 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.
- The median life expectancy for patients diagnosed with polycythemia vera exceeds 10 years.
- The prognosis of polycythemia vera is generally good with treatment. It will cause serious complications without treatment.
- Once myelofibrosis or acute myeloid leukemia ensues, the prognosis is very poor. Prognosis for post-PV myelofibrosis is adversely affected by older age, presence of leukocytosis, reticulin fibrosis, high JAK2 allele burden, splenomegaly, and history of venous thromboembolic events. 
- Post-PV acute myeloid leukemia, adverse risk factors include older age, leukocytosis, splenomegaly, reticulin, abnormal and complex karyotype, p53 mutation, and RUNX1 mutation.
- After transformation of polycythemia vera to myelofibrosis, the median survival is approximately 5.7 years.
- 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 27.8 years.
- There is a 4.2-fold increase in the risk of death in the presence of any of the following risk factors:
- 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.
- 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.
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