Materiały źródłowe

• #1 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #2 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  Polycythemia vera (PV), together with essential thrombocythemia (ET) and myelofibrosis (MF), belongs to the so-called “classic” BCR-ABL1-negative myeloproliferative neoplasms (MPN), a heterogeneous group of diseases, characterized by the clonal expansion of an abnormal hematopoietic stem/progenitor cell. Its incidence has been estimated to be 2.3–2.8 per 100,000 persons/year, with a median age at diagnosis of about 60 years and a male/female ratio of 1.2:1. […] As thrombotic events represent the main cause of morbidity and mortality for PV patients, with a registered rate of cardiovascular (CV) deaths and non-fatal thrombotic events of 5.5% patients/year, CV risk stratification is of crucial importance to evaluate patients’ prognosis at diagnosis. […] In the observational, prospective ECLAP study, which enrolled 1.638 PV patients, the global incidence of both arterial and venous thromboses was significantly higher among older patients (age > 65 years) or in the case of a previous thrombosis.

• #3 Polycythemia vera epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Polycythemia_vera_epidemiology_and_demographics

  The incidence of polycythemia vera is approximately 1.9 per 100,000 individuals per year in the US. The prevalence of polycythemia vera is 48 to 57 cases per 100,000 individuals in the United States. Males are more commonly affected than females. […] The incidence of polycythemia vera is about 1.9 per 100,000 individuals per year in the US. The incidence rate for men is 2.8 per 100,000 person-years, and is 1.3 per 100,000 person years for women. […] The prevalence of PV is estimated to be 44 to 57 cases per 100,000 persons, and approximately 148,000 persons are living with PV in the United States. […] The 15-year survival of patients with polycythemia vera is 65%. […] The mortality of polycythemia vera patients compared with the general population is 1.6-fold higher. […] The median age at diagnosis is 61 years but has been diagnosed in all age groups. The incidence increases with age. […] In ashkenazi jews, there is a high incidence of polycythemia vera. […] Males are more commonly affected with polycythemia vera than females. The male to female ratio is approximately 1.2 to 1.

• #4 Polycythemia vera – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/polycythemia-vera/

  Prevalence: approximately 44 per 100,000 population […] Age at diagnosis: variable; more common in individuals aged 60 years […] Risk factors for polycythemia vera include older age, male sex, white race, hypertension, diabetes mellitus, obesity, hyperlipidemia, and smoking. […] Epidemiological data refers to the US, unless otherwise specified.

• #5 Polycythemia vera epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Polycythemia_vera_epidemiology_and_demographics

  The incidence of polycythemia vera is approximately 1.9 per 100,000 individuals per year in the US. The prevalence of polycythemia vera is 48 to 57 cases per 100,000 individuals in the United States. Males are more commonly affected than females. […] The incidence of polycythemia vera is about 1.9 per 100,000 individuals per year in the US. The incidence rate for men is 2.8 per 100,000 person-years, and is 1.3 per 100,000 person years for women. […] The prevalence of PV is estimated to be 44 to 57 cases per 100,000 persons, and approximately 148,000 persons are living with PV in the United States. […] The 15-year survival of patients with polycythemia vera is 65%. […] The mortality of polycythemia vera patients compared with the general population is 1.6-fold higher. […] The median age at diagnosis is 61 years but has been diagnosed in all age groups. The incidence increases with age. […] In ashkenazi jews, there is a high incidence of polycythemia vera. […] Males are more commonly affected with polycythemia vera than females. The male to female ratio is approximately 1.2 to 1.

• #6 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #7 Clinical manifestations and diagnosis of polycythemia vera – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-polycythemia-vera

  Polycythemia vera (PV, polycythemia rubra vera, maladie de Vaquez) is one of the chronic myeloproliferative neoplasms (MPNs), which are collectively characterized by clonal proliferation of myeloid cells with variable morphologic maturity and hematopoietic efficiency. PV occurs in all populations, and all ages, including early adulthood and occasionally in children and adolescents. The median age at diagnosis is approximately 60 years. Approximately one-quarter of cases present before age 50 years and one-tenth before age 40 years. […] The incidence of PV in Olmsted County, Minnesota, during the period from 1935 through 1989, was estimated to be 1.9/100,000 per year, approximately one-half that for multiple myeloma in the same population. The incidence of PV is slightly higher in men than women (2.8 versus 1.3 cases/100,000 per year) and is highest for men aged 70 to 79 years (24 cases/100,000 persons per year). The incidence is lower in Japan than in the United States and Europe.

• #8 Polycythaemia Vera: Causes, Symptoms, and Treatment

  https://patient.info/doctor/polycythaemia-vera-pro

  Polycythaemia vera is rare:3 […] Registry-based studies from the European Union suggest an incidence of between 0.4 and 2.8 per 100,000 per year. […] A large study of US-based health plan data estimated a prevalence of 44 to 57 per 100,000. […] The reported median age at diagnosis ranges from 65-74 years.4 […] Familial cases are very rare and usually present in elderly patients.5

• #9

  https://link.springer.com/article/10.1007/s00277-025-06192-6

  There is little evidence, particularly in Germany, on the epidemiology and the cytoreductive management of polycythemia vera (PV). […] For the year 2021, we estimated a PV prevalence of 28.6 per 100,000 and an incidence of 3.3 per 100,000 in the German adult population (18 years). […] We identified 83.2% of prevalent patients in 2021 as being at high risk for thromboembolic complications, based on age (60 years) and/or history of TEs. […] Contrary to treatment guidelines, 43.6% of these high-risk patients did not receive cytoreductive drug treatment in 2021. […] Our findings suggest that currently available cytoreductive therapies are not being fully utilized according to treatment guidelines, which may lead to avoidable thromboembolic complications in this patient population. […] To our knowledge, epidemiologic data specifically for Germany are currently not available.

• #10 Polycythemia Vera Epidemiology Forecast 2023-2032: Cases Expected to Increase Across Regions and Genders – ResearchAndMarkets.com

  https://www.businesswire.com/news/home/20230320005394/en/Polycythemia-Vera-Epidemiology-Forecast-2023-2032-Cases-Expected-to-Increase-Across-Regions-and-Genders—ResearchAndMarkets.com

  Polycythemia Vera Epidemiology Forecast 2023-2032: Cases Expected to Increase Across Regions and Genders – ResearchAndMarkets.com […] In 2022, the prevalent cases of PV were highest in the US among the 7MM, which accounted for approximately 179,000 in 2022 and are expected to increase by 2032 […] According to the publisher estimates, there were around 124,000 cases of asymptomatic and 185,000 cases of symptomatic PV in the 7MM in 2022. The prevalence is projected to increase during the forecasted period […] In the United States, there were approximately 69,000 males and about 38,000 females affected with PV in 2022. These cases are likely to increase by 2032. […] The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by total prevalent cases of PV, prevalent population of PV based on symptoms, gender-specific cases of PV, prevalence of PV by gene mutation, prevalence of PV based on risk, and age-specific prevalence of PV, in the 7MM market covering the United States, EU4 countries (Germany, France, Italy, and Spain) the United Kingdom, and Japan from 2019 to 2032.

• #11 Polycythemia Vera Epidemiology Forecast 2023-2032: Cases Expected to Increase Across Regions and Genders – ResearchAndMarkets.com

  https://www.businesswire.com/news/home/20230320005394/en/Polycythemia-Vera-Epidemiology-Forecast-2023-2032-Cases-Expected-to-Increase-Across-Regions-and-Genders—ResearchAndMarkets.com

  The total prevalent cases of PV in the 7MM comprised of approximately 309,000 cases in 2022 and are projected to increase during the forecasted period […] The total diagnosed prevalent cases of PV in the United States were around 179,000 cases in 2022 […] The United States contributed to the largest prevalent population of PV, acquiring ~58% of the 7MM in 2022. Whereas, EU4 and the UK, and Japan accounted for around 32% and 10% of total population share, respectively, in 2022 […] According to the publisher estimates, there were around 72,000 cases of asymptomatic and 107,000 cases of symptomatic PV in the United States in 2022. The prevalence is projected to increase during the forecasted period […] In Japan, the prevalent PV cases were around 13,000 in males and 7,000 in females in 2022.

• #12 Clinical manifestations and diagnosis of polycythemia vera – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-polycythemia-vera

  Polycythemia vera (PV, polycythemia rubra vera, maladie de Vaquez) is one of the chronic myeloproliferative neoplasms (MPNs), which are collectively characterized by clonal proliferation of myeloid cells with variable morphologic maturity and hematopoietic efficiency. PV occurs in all populations, and all ages, including early adulthood and occasionally in children and adolescents. The median age at diagnosis is approximately 60 years. Approximately one-quarter of cases present before age 50 years and one-tenth before age 40 years. […] The incidence of PV in Olmsted County, Minnesota, during the period from 1935 through 1989, was estimated to be 1.9/100,000 per year, approximately one-half that for multiple myeloma in the same population. The incidence of PV is slightly higher in men than women (2.8 versus 1.3 cases/100,000 per year) and is highest for men aged 70 to 79 years (24 cases/100,000 persons per year). The incidence is lower in Japan than in the United States and Europe.

• #13 „Polycythemia Vera (PV): Epidemiology, Prognosis and Real-world Outcomes” and „Overcoming hypomethylating agent failure in acute myeloid leukemias and myelodysplastic syndromes” < Yale School of Medicine

  https://medicine.yale.edu/media-player/polycythemia-vera-pv-epidemiology-prognosis-and-real-world-outcomes-and-overcoming-hypomethylating-agent-failure-in-acute-myeloid-leukemias-and-myelodysplastic-syndromes/

  Polycythemia Vera Epidemiology was recently summarized in our review. As you can see the patients with this diagnosis are older, median age of diagnosis is 65 years. It’s not the most common malignancy. The incidence is only .5 to 400,000 person years. Estimated prevalence in the USA is 25 to 57 per 100,000 persons. Median overall survival is 12 to 14 years, which is less than expected in age and gender matched population. 5 year relative survival is 84 to 89%. […] The polycythemia Vera Epidemiology was recently summarized in our review. As you can see the patients with this diagnosis are older, median age of diagnosis is 65 years. It’s not the most common malignancy. The incidence is only .5 to 400,000 person years. Estimated prevalence in the USA is 25 to 57 per 100,000 persons. Median overall survival is 12 to 14 years, which is less than expected in age and gender matched population. 5 year relative survival is 84 to 89%.

• #14 Polycythemia vera – Wikipedia

  https://en.wikipedia.org/wiki/Polycythemia_vera

  Polycythemia vera occurs in all age groups, although the incidence increases with age. One study found the median age at diagnosis to be 60 years, while a Mayo Clinic study in Olmsted County, Minnesota found that the highest incidence was in people aged 70-79 years. The overall incidence in the Minnesota population was 1.9 per 100,000 person-years, and the disease was more common in men than women. […] A cluster around a toxic site was confirmed in northeast Pennsylvania in 2008.

• #15 Clinical manifestations and diagnosis of polycythemia vera – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-polycythemia-vera

  Polycythemia vera (PV, polycythemia rubra vera, maladie de Vaquez) is one of the chronic myeloproliferative neoplasms (MPNs), which are collectively characterized by clonal proliferation of myeloid cells with variable morphologic maturity and hematopoietic efficiency. PV occurs in all populations, and all ages, including early adulthood and occasionally in children and adolescents. The median age at diagnosis is approximately 60 years. Approximately one-quarter of cases present before age 50 years and one-tenth before age 40 years. […] The incidence of PV in Olmsted County, Minnesota, during the period from 1935 through 1989, was estimated to be 1.9/100,000 per year, approximately one-half that for multiple myeloma in the same population. The incidence of PV is slightly higher in men than women (2.8 versus 1.3 cases/100,000 per year) and is highest for men aged 70 to 79 years (24 cases/100,000 persons per year). The incidence is lower in Japan than in the United States and Europe.

• #16 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #17 Pediatric Polycythemia Vera: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/957470-overview

  The incidence of polycythemia vera is reported to be 4.9 cases per million population in Baltimore. A review of the disease in Connecticut reported an incidence of 22 cases per 100,000 population. […] The incidence of polycythemia vera is reported to be 6.7 cases per million population in Israel, and reviews have estimated 30 cases per 100,000 population in Sweden and Italy. In Norway, the prevalence of polycythemia vera is reported to be 9.2 cases per million inhabitants. […] Polycythemia vera is seen in every race. However, it appears to be more common in Jews of European extraction than in most non-Jewish populations. […] Males are preferentially affected over females, with the male-to-female ratio being 1.2-2.2:1. […] Onset is typically in the sixth decade, and the peak incidence is at age 60-80 years. A study from the Mayo Clinic reported that the incidence of polycythemia vera in persons aged 40 years or younger is about 12%.

• #18 Polycythemia vera epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Polycythemia_vera_epidemiology_and_demographics

  The incidence of polycythemia vera is approximately 1.9 per 100,000 individuals per year in the US. The prevalence of polycythemia vera is 48 to 57 cases per 100,000 individuals in the United States. Males are more commonly affected than females. […] The incidence of polycythemia vera is about 1.9 per 100,000 individuals per year in the US. The incidence rate for men is 2.8 per 100,000 person-years, and is 1.3 per 100,000 person years for women. […] The prevalence of PV is estimated to be 44 to 57 cases per 100,000 persons, and approximately 148,000 persons are living with PV in the United States. […] The 15-year survival of patients with polycythemia vera is 65%. […] The mortality of polycythemia vera patients compared with the general population is 1.6-fold higher. […] The median age at diagnosis is 61 years but has been diagnosed in all age groups. The incidence increases with age. […] In ashkenazi jews, there is a high incidence of polycythemia vera. […] Males are more commonly affected with polycythemia vera than females. The male to female ratio is approximately 1.2 to 1.

• #19 Polycythemia Vera – Market Insight, Epidemiology, and Market Forecast – 2034

  https://www.researchandmarkets.com/reports/5558047/polycythemia-vera-market-insight-epidemiology?srsltid=AfmBOoqm3qF-RvUkCNvb4MCBsffZwFozFxTUqC1NN3WZ-uigzmjXzlI8

  According to the publisher’s estimates, there were around 72,810 cases of asymptomatic and 109,210 cases of symptomatic Polycythemia Vera in the United States in 2024. The prevalence is projected to increase during the forecasted period. […] In 7MM, approximately 56% of the patient share is attributed to males, whereas only 44% of females suffer from Polycythemia Vera.

• #20 Polycythemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK526081/

  The prevalence of polycythemia vera has been estimated to be approximately 22 cases per 100,000 population. It is believed to occur more frequently among Jewish patients of Eastern European descent than other Europeans and Asians. Polycythemia vera shows a male preponderance in all races and ethnicities, with a male-to-female ratio of approximately 2 to 1. The median age of presentation of PV is 60 years, with patients seldom seen before the age of 40. Polycythemia due to hemoglobinopathies and congenital cyanotic heart diseases is likely to be detected in significantly younger patients.

• #21 Pediatric Polycythemia Vera: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/957470-overview

  The incidence of polycythemia vera is reported to be 4.9 cases per million population in Baltimore. A review of the disease in Connecticut reported an incidence of 22 cases per 100,000 population. […] The incidence of polycythemia vera is reported to be 6.7 cases per million population in Israel, and reviews have estimated 30 cases per 100,000 population in Sweden and Italy. In Norway, the prevalence of polycythemia vera is reported to be 9.2 cases per million inhabitants. […] Polycythemia vera is seen in every race. However, it appears to be more common in Jews of European extraction than in most non-Jewish populations. […] Males are preferentially affected over females, with the male-to-female ratio being 1.2-2.2:1. […] Onset is typically in the sixth decade, and the peak incidence is at age 60-80 years. A study from the Mayo Clinic reported that the incidence of polycythemia vera in persons aged 40 years or younger is about 12%.

• #22 The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia | Experimental Hematology & Oncology | Full Text

  https://ehoonline.biomedcentral.com/articles/10.1186/s40164-018-0124-7

  The epidemiology is well described in the western world but not in Asian countries like Malaysia. […] A total of 1010 patients were registered over a period of 5 years. […] The types of MPN reported were 40.4% of ET (n=408), 38.1% of PV (n=385), 9.2% of PMF (n=93), 3.1% of hypereosinophilic syndrome (HES) (n=31) and 7.9% of unclassifiable MPN (MPN-U) (n=80). […] The presence of JAK2 V617F mutation is associated with higher risk of arterial thrombosis. […] Chinese ethnicity is associated with higher rates of MPN. […] This study is instrumental for policy makers to ensure preventive strategies can be implemented in future. […] The development of JAK2 V617F inhibitor, ruxolitinib as the treatment for those with JAK2 V617F myelofibrosis has not been as successful as those CML with TKI.

• #23 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #24 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #25 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #26 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #27 Take a look at the Recent articles

  https://www.oatext.com/Immunotherapy-and-polycythemia-vera.php

  Petrochemicals such as benzene and toluene, and ionizing radiation increase the risk of PV and PMF. […] The identification of the JAK2V617F mutation in patients diagnosed and progressing to chronic MPDs has stimulated a great deal of effort in screening for MPDs and for developing specific JAK1/2 and other inhibitors for clinical therapy.

• #28 Take a look at the Recent articles

  https://www.oatext.com/Immunotherapy-and-polycythemia-vera.php

  Polycythemia vera (PV) is a myeloproliferative disorder, which presents as an abnormal increase in the number of red blood cells. It is the most common form of the MPDs. Its incidence in the US is estimated to be 1.9/100,000, with an increase associated with ageing. The mean age at diagnosis of PV is approximately 60 years old. However, in 2010 the age adjusted prevalence of PV was 57.15/100,000. PV is very rare in children. […] The cause of chronic MPDs remains unknown. A mutation in a specific protein, Janus kinase 2 (JAK2) is found in a large number of people with MPDs. It is detected in about 95% of those with PV. […] Although one third of the cases diagnosed are in those below the age of 50, PV is most commonly diagnosed at 60 years of age and above. […] PV, as well as all MPDs in general, are more prevalent in men.

• #29 Polycythemia Vera (PV): Trends, Epidemiology (2017-2030),

  https://www.globenewswire.com/news-release/2020/07/15/2062391/0/en/Polycythemia-Vera-PV-Trends-Epidemiology-2017-2030-KOL-Views-and-Unmet-Needs.html

  It has been also observed that PV in the US is mostly prevalent in the age group of 75 years. […] A total of 94,374 diagnosed (symptomatic) prevalent population of PV was assessed in 2017. Out of these cases, ~87,881 cases of PV were calculated for patients with JAK2 V617F, thereby accounting for the highest number of cases with gene mutation with ~97% of total PV cases with JAK2 mutation. […] Around 23% of patients accounted for low-risk categories, and 77% accounted for high-risk cases in 2017 in the United States.

• #30 Clinical manifestations and diagnosis of polycythemia vera – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-polycythemia-vera

  While there is no known familial disposition to PV, rare families have been described in which multiple members develop myeloproliferative neoplasms, including PV. Studies of these families suggest the presence of an autosomal dominant mutation that may predispose to acquisition of a secondary somatic mutation such as a JAK2 mutation.

• #31 Polycythemia Vera – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557660/

  Polycythemia vera (PV) can affect all ethnic groups with no sex predilection, although there are slightly more cases in men than women. It can occur in all age groups, but the median age of diagnosis is 60. PV affects 0.6 to 1.6 per million people in the United States. There are fewer incidences in Japan than in the United States or Europe. […] The average survival of untreated polycythemia vera (PV) is 18 months, whereas median survival is 14 years overall and 24 years if younger than 60 for those undergoing treatment. According to one study, mortality in PV is higher than in age and sex-matched populations. Five-year survival in the overall cohort was 79.5%, but patients are at a high risk of second primary malignancies and leukemic transformation, which may compromise long-term survival.

• #32 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #33 Polycythemia Vera – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557660/

  Polycythemia vera (PV) can affect all ethnic groups with no sex predilection, although there are slightly more cases in men than women. It can occur in all age groups, but the median age of diagnosis is 60. PV affects 0.6 to 1.6 per million people in the United States. There are fewer incidences in Japan than in the United States or Europe. […] The average survival of untreated polycythemia vera (PV) is 18 months, whereas median survival is 14 years overall and 24 years if younger than 60 for those undergoing treatment. According to one study, mortality in PV is higher than in age and sex-matched populations. Five-year survival in the overall cohort was 79.5%, but patients are at a high risk of second primary malignancies and leukemic transformation, which may compromise long-term survival.

• #34 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #35 Polycythemia Vera – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557660/

  Polycythemia vera (PV) can affect all ethnic groups with no sex predilection, although there are slightly more cases in men than women. It can occur in all age groups, but the median age of diagnosis is 60. PV affects 0.6 to 1.6 per million people in the United States. There are fewer incidences in Japan than in the United States or Europe. […] The average survival of untreated polycythemia vera (PV) is 18 months, whereas median survival is 14 years overall and 24 years if younger than 60 for those undergoing treatment. According to one study, mortality in PV is higher than in age and sex-matched populations. Five-year survival in the overall cohort was 79.5%, but patients are at a high risk of second primary malignancies and leukemic transformation, which may compromise long-term survival.

• #36 „Polycythemia Vera (PV): Epidemiology, Prognosis and Real-world Outcomes” and „Overcoming hypomethylating agent failure in acute myeloid leukemias and myelodysplastic syndromes” < Yale School of Medicine

  https://medicine.yale.edu/media-player/polycythemia-vera-pv-epidemiology-prognosis-and-real-world-outcomes-and-overcoming-hypomethylating-agent-failure-in-acute-myeloid-leukemias-and-myelodysplastic-syndromes/

  Polycythemia Vera Epidemiology was recently summarized in our review. As you can see the patients with this diagnosis are older, median age of diagnosis is 65 years. It’s not the most common malignancy. The incidence is only .5 to 400,000 person years. Estimated prevalence in the USA is 25 to 57 per 100,000 persons. Median overall survival is 12 to 14 years, which is less than expected in age and gender matched population. 5 year relative survival is 84 to 89%. […] The polycythemia Vera Epidemiology was recently summarized in our review. As you can see the patients with this diagnosis are older, median age of diagnosis is 65 years. It’s not the most common malignancy. The incidence is only .5 to 400,000 person years. Estimated prevalence in the USA is 25 to 57 per 100,000 persons. Median overall survival is 12 to 14 years, which is less than expected in age and gender matched population. 5 year relative survival is 84 to 89%.

• #37 Polycythemia Vera: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0601/p680.html

  The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000. Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age. Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent. Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia. […] Smoking cessation decreases the risk of thrombosis. Without treatment, death typically occurs within two years, mostly from thrombotic events. Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years. For those diagnosed before 60 years of age, median survival is 24 years. The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%. Progression to myelofibrosis over 15 years is 6% to 14%.

• #38 Polycythemia vera epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Polycythemia_vera_epidemiology_and_demographics

  The incidence of polycythemia vera is approximately 1.9 per 100,000 individuals per year in the US. The prevalence of polycythemia vera is 48 to 57 cases per 100,000 individuals in the United States. Males are more commonly affected than females. […] The incidence of polycythemia vera is about 1.9 per 100,000 individuals per year in the US. The incidence rate for men is 2.8 per 100,000 person-years, and is 1.3 per 100,000 person years for women. […] The prevalence of PV is estimated to be 44 to 57 cases per 100,000 persons, and approximately 148,000 persons are living with PV in the United States. […] The 15-year survival of patients with polycythemia vera is 65%. […] The mortality of polycythemia vera patients compared with the general population is 1.6-fold higher. […] The median age at diagnosis is 61 years but has been diagnosed in all age groups. The incidence increases with age. […] In ashkenazi jews, there is a high incidence of polycythemia vera. […] Males are more commonly affected with polycythemia vera than females. The male to female ratio is approximately 1.2 to 1.

• #39

  https://step1.medbullets.com/oncology/117019/polycythemia-vera

  Epidemiology […] presentation in those aged 60-70 […] 1.6x higher mortality than general population

• #40 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  Polycythemia vera (PV), together with essential thrombocythemia (ET) and myelofibrosis (MF), belongs to the so-called “classic” BCR-ABL1-negative myeloproliferative neoplasms (MPN), a heterogeneous group of diseases, characterized by the clonal expansion of an abnormal hematopoietic stem/progenitor cell. Its incidence has been estimated to be 2.3–2.8 per 100,000 persons/year, with a median age at diagnosis of about 60 years and a male/female ratio of 1.2:1. […] As thrombotic events represent the main cause of morbidity and mortality for PV patients, with a registered rate of cardiovascular (CV) deaths and non-fatal thrombotic events of 5.5% patients/year, CV risk stratification is of crucial importance to evaluate patients’ prognosis at diagnosis. […] In the observational, prospective ECLAP study, which enrolled 1.638 PV patients, the global incidence of both arterial and venous thromboses was significantly higher among older patients (age > 65 years) or in the case of a previous thrombosis.

• #41 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  Polycythemia vera (PV), together with essential thrombocythemia (ET) and myelofibrosis (MF), belongs to the so-called “classic” BCR-ABL1-negative myeloproliferative neoplasms (MPN), a heterogeneous group of diseases, characterized by the clonal expansion of an abnormal hematopoietic stem/progenitor cell. Its incidence has been estimated to be 2.3–2.8 per 100,000 persons/year, with a median age at diagnosis of about 60 years and a male/female ratio of 1.2:1. […] As thrombotic events represent the main cause of morbidity and mortality for PV patients, with a registered rate of cardiovascular (CV) deaths and non-fatal thrombotic events of 5.5% patients/year, CV risk stratification is of crucial importance to evaluate patients’ prognosis at diagnosis. […] In the observational, prospective ECLAP study, which enrolled 1.638 PV patients, the global incidence of both arterial and venous thromboses was significantly higher among older patients (age > 65 years) or in the case of a previous thrombosis.

• #42 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  Currently, patients are stratified into a low-risk group, in the case of younger age (<60 years) and no previous thromboses, and a high-risk group, in the case of patients older than 60 years and/or with a previous thrombotic complication. [...] Generic CV risk factors, particularly hypertension, smoking, and leukocytosis, while not yet formally included in a risk scoring system, contribute, as one can imagine, to the overall risk of thrombosis. [...] The main reason for these changes was represented by the recognition of a new entity, namely the so-called “masked PV”; indeed, individuals suffering from this condition have a worse outcome, possibly owing to missed or delayed diagnoses and, as a consequence, a lower intensity of treatment.

• #43 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  Currently, patients are stratified into a low-risk group, in the case of younger age (<60 years) and no previous thromboses, and a high-risk group, in the case of patients older than 60 years and/or with a previous thrombotic complication. [...] Generic CV risk factors, particularly hypertension, smoking, and leukocytosis, while not yet formally included in a risk scoring system, contribute, as one can imagine, to the overall risk of thrombosis. [...] The main reason for these changes was represented by the recognition of a new entity, namely the so-called “masked PV”; indeed, individuals suffering from this condition have a worse outcome, possibly owing to missed or delayed diagnoses and, as a consequence, a lower intensity of treatment.

• #44

  https://link.springer.com/article/10.1007/s00277-025-06192-6

  Our estimate for 2021 of 28.6 per 100,000 is also at the upper end of this range. […] We considered most prevalent patients in 2021 (83.2%) to have high-risk PV, either due to their age (60 years in 2021) or their recorded history of TEs (criterion checked between 2019 and 2021), which is in line with previous observational studies in which the majority of patients were categorized as high-risk, mainly due to their age. […] Contrary to current treatment guideline recommendations, 43.6% of the patients we categorized as high-risk patients in 2021 did not receive treatment with cytoreductive drugs in that year. […] In this context, much emphasis has been placed on control of the hematocrit below 45% as this target level has been associated with significantly lower rates of major TEs and death.

• #45

  https://link.springer.com/article/10.1007/s00277-025-06192-6

  Our estimate for 2021 of 28.6 per 100,000 is also at the upper end of this range. […] We considered most prevalent patients in 2021 (83.2%) to have high-risk PV, either due to their age (60 years in 2021) or their recorded history of TEs (criterion checked between 2019 and 2021), which is in line with previous observational studies in which the majority of patients were categorized as high-risk, mainly due to their age. […] Contrary to current treatment guideline recommendations, 43.6% of the patients we categorized as high-risk patients in 2021 did not receive treatment with cytoreductive drugs in that year. […] In this context, much emphasis has been placed on control of the hematocrit below 45% as this target level has been associated with significantly lower rates of major TEs and death.

• #46 New Perspectives on Polycythemia Vera: From Diagnosis to Therapy

  https://www.mdpi.com/1422-0067/21/16/5805

  Currently, patients are stratified into a low-risk group, in the case of younger age (<60 years) and no previous thromboses, and a high-risk group, in the case of patients older than 60 years and/or with a previous thrombotic complication. [...] Generic CV risk factors, particularly hypertension, smoking, and leukocytosis, while not yet formally included in a risk scoring system, contribute, as one can imagine, to the overall risk of thrombosis. [...] The main reason for these changes was represented by the recognition of a new entity, namely the so-called “masked PV”; indeed, individuals suffering from this condition have a worse outcome, possibly owing to missed or delayed diagnoses and, as a consequence, a lower intensity of treatment.

• #47 ATSDR – Polycythemia Vera Investigation – Current Projects

  https://archive.cdc.gov/www_atsdr_cdc_gov/sites/polycythemia_vera/projects.html

  Polycythemia Vera Investigation portfolio Epidemiology […] This study compared the pattern of PV in an area of Pennsylvania where people have similar characteristics (such as age and race) to people in the tri-county/cluster area. […] This study aimed to improve physicians reporting of PV and other blood cancers to central cancer registries. […] Using medical records of patients with a diagnosis of PV, this study determined if the diagnosis was correct, if the cases were reported to the cancer registry, and if the doctor used the JAK2V617F test to help diagnose PV. […] This study aimed to identify risk factors that might be linked to developing PV, ET, and PMF for persons living in the cluster area. […] This surveillance study aimed to determine how complete and accurate reporting to the Pennsylvania Cancer Registry is in the tri-county area for MPNs (specifically, PV, ET, PMF, chronic myelogenous leukemia, and MPN, not otherwise specified).

• #48 Incidence of Myeloproliferative Neoplasms: Population-based, US Data – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/incidence-myeloproliferative-neoplasms-population-based-us-data/1155

  Until recently, US single-center and European population-based studies provided most of what was known about the epidemiology of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV). […] Last year, research funded by the National Institute of Health helped to disclose extensive epidemiological trends of MPNs between 2001 and 2012. […] Mining data from the Surveillance, Epidemiology and End Results (SEER) Program, which encompassed 18 registries and 28% of the US population, researchers analyzed the incidence rate (IR) and relative survival (RS) of malignant MPN subtypes. […] This is the largest population-based study of MPNs and [myelodysplastic]/MPNs in the US that comprehensively describes incidence patterns and patient survival by disease subtypes. […] PV had the highest incidence rate (IR) of the MPNs analyzed (IR=10.9), followed by essential thrombocythemia (ET) (IR=9.6).

• #49 Incidence of Myeloproliferative Neoplasms: Population-based, US Data – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/incidence-myeloproliferative-neoplasms-population-based-us-data/1155

  Until recently, US single-center and European population-based studies provided most of what was known about the epidemiology of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV). […] Last year, research funded by the National Institute of Health helped to disclose extensive epidemiological trends of MPNs between 2001 and 2012. […] Mining data from the Surveillance, Epidemiology and End Results (SEER) Program, which encompassed 18 registries and 28% of the US population, researchers analyzed the incidence rate (IR) and relative survival (RS) of malignant MPN subtypes. […] This is the largest population-based study of MPNs and [myelodysplastic]/MPNs in the US that comprehensively describes incidence patterns and patient survival by disease subtypes. […] PV had the highest incidence rate (IR) of the MPNs analyzed (IR=10.9), followed by essential thrombocythemia (ET) (IR=9.6).

• #50 Prevalence, incidence, and thromboembolic events in polycythemia vera: a study based on longitudinal German health claims data | springermedizin.de

  https://www.springermedizin.de/prevalence-incidence-and-thromboembolic-events-in-polycythemia-v/50616710

  There is little evidence, particularly in Germany, on the epidemiology and the cytoreductive management of polycythemia vera (PV). […] For the year 2021, we estimated a PV prevalence of 28.6 per 100,000 and an incidence of 3.3 per 100,000 in the German adult population (18 years). […] Our findings suggest that currently available cytoreductive therapies are not being fully utilized according to treatment guidelines, which may lead to avoidable thromboembolic complications in this patient population. […] To our knowledge, epidemiologic data specifically for Germany are currently not available. […] Our estimate for 2021 of 28.6 per 100,000 is also at the upper end of this range. […] Our prevalence estimates were also slightly higher in 2020 and 2021, when phlebotomy therapy could be included as a criterion to validate prevalent case identification.

• #51 Polycythemia vera – Wikipedia

  https://en.wikipedia.org/wiki/Polycythemia_vera

  Polycythemia vera occurs in all age groups, although the incidence increases with age. One study found the median age at diagnosis to be 60 years, while a Mayo Clinic study in Olmsted County, Minnesota found that the highest incidence was in people aged 70-79 years. The overall incidence in the Minnesota population was 1.9 per 100,000 person-years, and the disease was more common in men than women. […] A cluster around a toxic site was confirmed in northeast Pennsylvania in 2008.

• #52 ATSDR – Polycythemia Vera Investigation – Current Projects

  https://archive.cdc.gov/www_atsdr_cdc_gov/sites/polycythemia_vera/projects.html

  Polycythemia Vera Investigation portfolio Epidemiology […] This study compared the pattern of PV in an area of Pennsylvania where people have similar characteristics (such as age and race) to people in the tri-county/cluster area. […] This study aimed to improve physicians reporting of PV and other blood cancers to central cancer registries. […] Using medical records of patients with a diagnosis of PV, this study determined if the diagnosis was correct, if the cases were reported to the cancer registry, and if the doctor used the JAK2V617F test to help diagnose PV. […] This study aimed to identify risk factors that might be linked to developing PV, ET, and PMF for persons living in the cluster area. […] This surveillance study aimed to determine how complete and accurate reporting to the Pennsylvania Cancer Registry is in the tri-county area for MPNs (specifically, PV, ET, PMF, chronic myelogenous leukemia, and MPN, not otherwise specified).

• #53 ATSDR – Polycythemia Vera Investigation – Current Projects

  https://archive.cdc.gov/www_atsdr_cdc_gov/sites/polycythemia_vera/projects.html

  Polycythemia Vera Investigation portfolio Epidemiology […] This study compared the pattern of PV in an area of Pennsylvania where people have similar characteristics (such as age and race) to people in the tri-county/cluster area. […] This study aimed to improve physicians reporting of PV and other blood cancers to central cancer registries. […] Using medical records of patients with a diagnosis of PV, this study determined if the diagnosis was correct, if the cases were reported to the cancer registry, and if the doctor used the JAK2V617F test to help diagnose PV. […] This study aimed to identify risk factors that might be linked to developing PV, ET, and PMF for persons living in the cluster area. […] This surveillance study aimed to determine how complete and accurate reporting to the Pennsylvania Cancer Registry is in the tri-county area for MPNs (specifically, PV, ET, PMF, chronic myelogenous leukemia, and MPN, not otherwise specified).

• #54 Polycythemia Vera: Geographic Clustering, Genetic Link? – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/polycythemia-vera-geographic-clustering-genetic-link/2643

  Polycythemia vera (PV) is a rare, chronic clonal hematologic malignancy predominately characterized by an increased red blood cell mass and defined by the presence of a Janus kinase 2 (JAK2) mutation. […] A recent study analyzed patient demographics, incidence, and geographic distribution of PV patients in Canada during a 19-year period. […] Significantly higher incidence rates compared with the national average were noted in both the province of Quebec and in the western provinces of Manitoba, Saskatchewan, Alberta, and British Columbia, areas with larger Jewish communities. […] Understanding of the epidemiology and pathogenesis of PV can lead to increased awareness of important risk factors, according to the authors of a recent epidemiological analysis published in Cancer. […] Their analysis included a total of 4645 patients who were diagnosed with PV in Canada in that timeframe; about half were men (50.4%), and the mean age at diagnosis was 68.8 years, demographics that are akin to those noted in the United States.

• #55 Polycythemia Vera: Geographic Clustering, Genetic Link? – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/polycythemia-vera-geographic-clustering-genetic-link/2643

  Interestingly, significantly higher incidence rates as compared with the national average were noted in both the province of Quebec and in the western provinces of Manitoba, Saskatchewan, Alberta, and British Columbia. […] Most strikingly, a geographic clustering of PV incidence was noted in the Côute-Saint-Luc borough of Montreal, Quebec. […] During the 19-year study period, 45 cases of PV were documented in Côute-Saint-Luc, representing a 13-fold increase in incidence compared with the national average. […] Significantly higher incidence rates compared with the national average were also found in the West Island, Ville. St. Laurent, and Côute-des-Neiges boroughs in Montreal—other boroughs with large ethnically Jewish populations. […] The authors speculated that fact may also have contributed to the higher incidence of PV in that region, given the increased risk of PV with advanced age. […] This study provides an extensive analysis of the demographics and epidemiology of PV incidence and mortality in Canada.

• #56 Polycythemia Vera: Geographic Clustering, Genetic Link? – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/polycythemia-vera-geographic-clustering-genetic-link/2643

  Interestingly, significantly higher incidence rates as compared with the national average were noted in both the province of Quebec and in the western provinces of Manitoba, Saskatchewan, Alberta, and British Columbia. […] Most strikingly, a geographic clustering of PV incidence was noted in the Côute-Saint-Luc borough of Montreal, Quebec. […] During the 19-year study period, 45 cases of PV were documented in Côute-Saint-Luc, representing a 13-fold increase in incidence compared with the national average. […] Significantly higher incidence rates compared with the national average were also found in the West Island, Ville. St. Laurent, and Côute-des-Neiges boroughs in Montreal—other boroughs with large ethnically Jewish populations. […] The authors speculated that fact may also have contributed to the higher incidence of PV in that region, given the increased risk of PV with advanced age. […] This study provides an extensive analysis of the demographics and epidemiology of PV incidence and mortality in Canada.

• #57 Potential limitations of diagnostic standard codes to distinguish polycythemia vera and secondary erythrocytosis | Scientific Reports

  https://www.nature.com/articles/s41598-022-08606-1

  Red cell overproduction is seen in polycythemia vera (PV), a bone marrow myeloproliferative neoplasm characterized by trilinear cell proliferation (WBC, platelets), as well as in secondary erythrocytosis (SE), a group of heterogeneous disorders characterized by elevated EPO gene transcription. […] The prevalence of PV is estimated to be 4755 per 100,000 in the United States. […] Despite the expertise and forward understanding of pathophysiology, for some cases, the classification and management of the disease can be far from straightforward and challenging. […] The impact of erroneous adjudication of diagnostic codes may have an impact on interventions using data from public health registries, surveillance and disease control at a population level. […] The numerous variables, the high number of unexplained cases reported, as shown in a cross-sectional study from NHANES 2007-2008 and the misidentification of erythrocytosis seen in some cases, make data collection complex.

• #58 Potential limitations of diagnostic standard codes to distinguish polycythemia vera and secondary erythrocytosis | Scientific Reports

  https://www.nature.com/articles/s41598-022-08606-1

  Red cell overproduction is seen in polycythemia vera (PV), a bone marrow myeloproliferative neoplasm characterized by trilinear cell proliferation (WBC, platelets), as well as in secondary erythrocytosis (SE), a group of heterogeneous disorders characterized by elevated EPO gene transcription. […] The prevalence of PV is estimated to be 4755 per 100,000 in the United States. […] Despite the expertise and forward understanding of pathophysiology, for some cases, the classification and management of the disease can be far from straightforward and challenging. […] The impact of erroneous adjudication of diagnostic codes may have an impact on interventions using data from public health registries, surveillance and disease control at a population level. […] The numerous variables, the high number of unexplained cases reported, as shown in a cross-sectional study from NHANES 2007-2008 and the misidentification of erythrocytosis seen in some cases, make data collection complex.

• #59 Potential limitations of diagnostic standard codes to distinguish polycythemia vera and secondary erythrocytosis | Scientific Reports

  https://www.nature.com/articles/s41598-022-08606-1

  Challenges of diagnosis between etiologies of erythrocytosis (PV versus SE) could be clarified after JAK2 somatic mutation genotyping is performed, as it prevails as a determinant tool in diagnosis. […] The use of ICD data capture modalities is critical to accurately identify specific populations of interest to conduct retrospective research; however, limitations are associated with possible misdiagnosed cases. […] Research based exclusively on ICD codes could have a potential impact on public health and patient care, and limitations must be weighed when research findings are conveyed.

• #60 Potential limitations of diagnostic standard codes to distinguish polycythemia vera and secondary erythrocytosis | Scientific Reports

  https://www.nature.com/articles/s41598-022-08606-1

  Challenges of diagnosis between etiologies of erythrocytosis (PV versus SE) could be clarified after JAK2 somatic mutation genotyping is performed, as it prevails as a determinant tool in diagnosis. […] The use of ICD data capture modalities is critical to accurately identify specific populations of interest to conduct retrospective research; however, limitations are associated with possible misdiagnosed cases. […] Research based exclusively on ICD codes could have a potential impact on public health and patient care, and limitations must be weighed when research findings are conveyed.

• #61 Potential limitations of diagnostic standard codes to distinguish polycythemia vera and secondary erythrocytosis | Scientific Reports

  https://www.nature.com/articles/s41598-022-08606-1

  Challenges of diagnosis between etiologies of erythrocytosis (PV versus SE) could be clarified after JAK2 somatic mutation genotyping is performed, as it prevails as a determinant tool in diagnosis. […] The use of ICD data capture modalities is critical to accurately identify specific populations of interest to conduct retrospective research; however, limitations are associated with possible misdiagnosed cases. […] Research based exclusively on ICD codes could have a potential impact on public health and patient care, and limitations must be weighed when research findings are conveyed.

• #62 Polycythemia Vera: Geographic Clustering, Genetic Link? – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/polycythemia-vera-geographic-clustering-genetic-link/2643

  Polycythemia vera (PV) is a rare, chronic clonal hematologic malignancy predominately characterized by an increased red blood cell mass and defined by the presence of a Janus kinase 2 (JAK2) mutation. […] A recent study analyzed patient demographics, incidence, and geographic distribution of PV patients in Canada during a 19-year period. […] Significantly higher incidence rates compared with the national average were noted in both the province of Quebec and in the western provinces of Manitoba, Saskatchewan, Alberta, and British Columbia, areas with larger Jewish communities. […] Understanding of the epidemiology and pathogenesis of PV can lead to increased awareness of important risk factors, according to the authors of a recent epidemiological analysis published in Cancer. […] Their analysis included a total of 4645 patients who were diagnosed with PV in Canada in that timeframe; about half were men (50.4%), and the mean age at diagnosis was 68.8 years, demographics that are akin to those noted in the United States.

• #63 The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia | Experimental Hematology & Oncology | Full Text

  https://ehoonline.biomedcentral.com/articles/10.1186/s40164-018-0124-7

  The epidemiology is well described in the western world but not in Asian countries like Malaysia. […] A total of 1010 patients were registered over a period of 5 years. […] The types of MPN reported were 40.4% of ET (n=408), 38.1% of PV (n=385), 9.2% of PMF (n=93), 3.1% of hypereosinophilic syndrome (HES) (n=31) and 7.9% of unclassifiable MPN (MPN-U) (n=80). […] The presence of JAK2 V617F mutation is associated with higher risk of arterial thrombosis. […] Chinese ethnicity is associated with higher rates of MPN. […] This study is instrumental for policy makers to ensure preventive strategies can be implemented in future. […] The development of JAK2 V617F inhibitor, ruxolitinib as the treatment for those with JAK2 V617F myelofibrosis has not been as successful as those CML with TKI.

• #64 The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia | Experimental Hematology & Oncology | Full Text

  https://ehoonline.biomedcentral.com/articles/10.1186/s40164-018-0124-7

  The epidemiology is well described in the western world but not in Asian countries like Malaysia. […] A total of 1010 patients were registered over a period of 5 years. […] The types of MPN reported were 40.4% of ET (n=408), 38.1% of PV (n=385), 9.2% of PMF (n=93), 3.1% of hypereosinophilic syndrome (HES) (n=31) and 7.9% of unclassifiable MPN (MPN-U) (n=80). […] The presence of JAK2 V617F mutation is associated with higher risk of arterial thrombosis. […] Chinese ethnicity is associated with higher rates of MPN. […] This study is instrumental for policy makers to ensure preventive strategies can be implemented in future. […] The development of JAK2 V617F inhibitor, ruxolitinib as the treatment for those with JAK2 V617F myelofibrosis has not been as successful as those CML with TKI.

• #65 Incidence of Myeloproliferative Neoplasms: Population-based, US Data – Focus on Polycythemia Vera & Related Disorders

  https://www.medpagetoday.com/resource-centers/polycythemia-vera/incidence-myeloproliferative-neoplasms-population-based-us-data/1155

  Compared to older patients, younger patients (<60 years) had better 5-year relative survival (RS) for all MPNs. [...] The highest incidence of PV occurred in calendar year 2003-2004, with rates decreasing steadily thereafter. [...] The findings highlighted the impact of molecular testing on clinical care. [...] The joint availability of molecular testing and effective targeted therapies is likely to increase awareness of the diagnoses of MPNs.

• #66 Systematization of analytical studies of polycythemia vera, essential thrombocythemia and primary myelofibrosis, and a meta-analysis of the frequency of JAK2, CALR and MPL mutations: 2000–2018 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-5764-4

  The incidence of PV per 100,000 inhabitants is 0.84 at the global level, and in Europe and North America it has been registered at 0.86 and 0.74 respectively. […] It is necessary to mention that many studies have been carried out in high-income countries, so it is probable that in middle-income and low-income countries, MPN is misclassified and the incidences may be underestimated. […] A total of 27,078 study subjects were included, of which 12.9% (n=3488) corresponded to patients with PV, 19.6% (n=5300) with ET, 7.2% (n=1954) with PMF and 60.3% to healthy controls (n=1070) and people with other diseases (n=15,266). […] The results of the studies showed a high degree of heterogeneity. […] The meta-analysis showed that the probability of presenting JAK2V617F mutation in patients with PV is 3.0 times higher than for ET and 4.0 times higher than PMF.

• #67 Systematization of analytical studies of polycythemia vera, essential thrombocythemia and primary myelofibrosis, and a meta-analysis of the frequency of JAK2, CALR and MPL mutations: 2000–2018 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-5764-4

  The MPL mutation was analyzed in 14 studies, and presented with the frequencies 0% in PV, from 0.9 to 12.5% in ET, and from 0 to 17.1% in PMF. […] The CALR mutation was reported in 13 studies, with a frequency of 0.0% in PV. In ET it ranged from 12.6 to 50% and in PMF from 10 to 100%. […] Given their specificity and high reported frequencies in this group of neoplasms, the diagnosis of these entities should not be made only by clinical and hematological characteristics alone but also by the genetic screening of patients.

• #68 Systematization of analytical studies of polycythemia vera, essential thrombocythemia and primary myelofibrosis, and a meta-analysis of the frequency of JAK2, CALR and MPL mutations: 2000–2018 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-5764-4

  The MPL mutation was analyzed in 14 studies, and presented with the frequencies 0% in PV, from 0.9 to 12.5% in ET, and from 0 to 17.1% in PMF. […] The CALR mutation was reported in 13 studies, with a frequency of 0.0% in PV. In ET it ranged from 12.6 to 50% and in PMF from 10 to 100%. […] Given their specificity and high reported frequencies in this group of neoplasms, the diagnosis of these entities should not be made only by clinical and hematological characteristics alone but also by the genetic screening of patients.

• #69 Polycythemia Vera Life Expectancy: Benefits of Treatment

  https://www.verywellhealth.com/polycythemia-vera-life-expectancy-5211969

  Polycythemia vera can be treated with medical interventions and medication to reduce the effects of complications and improve life expectancy. Since there is no cure, disease surveillance and treatment have to be continued long term. […] Polycythemia vera can lower a persons life expectancy due to complications, including blood clots that can affect the heart, brain, lungs, or other organs. These effects may cause lasting disability and can be fatal. Survival rates have been increasing due to improved treatments. […] With treatment, the median survival rate is approximately 15 years after diagnosis, and for patients aged 40 or younger, median survival is 37 years after diagnosis. […] Polycythemia vera and secondary polycythemia are conditions in which the body makes too many red blood cells. These conditions need to be treated because they can cause serious complications and may affect survival. With treatment, life expectancy is substantially improved. […] Treatment and surveillance of the effects of your disease must be consistent and lifelong.

• #70

  https://link.springer.com/article/10.1007/s00277-025-06192-6

  Currently, there is strong consensus that patients with high-risk PV should be treated with cytoreductive drugs to reduce TE risk by controlling blood cell count. […] Our results indicate that, contrary to guideline recommendations, there might be a number of patients whose treatment is not adjusted despite showing signs of potential HU resistance/intolerance. […] This raises concerns because intolerance and resistance to HU have been linked to adverse outcomes, including an increased risk of thrombosis, lower survival rates, and higher chances of disease progression. […] Our analysis was not adjusted for differences in the composition of the cohorts (e.g., in terms age, CVRFs, treatment status [prevalent vs. new-user]). […] Our findings suggest that currently available cytoreductive therapies are not being fully utilized according to treatment guidelines.

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