Materiały źródłowe

• #1 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). […] Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P<0.001). [...] In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0).

• #2 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. […] Current treatment in PV has not affected the natural history of the disease in regards to overall, leukemia-free or myelofibrosis-free survival, but thrombosis-free survival has been positively affected by treatment with phlebotomy, aspirin and cytoreductive drugs. […] Survival in PV is inferior to that of ET but superior to that of PMF, with estimated medians of 14, 20, and 6 years, respectively; the corresponding figures for patients younger than age 60 years are 24, 33, and 15 years. […] In the largest international study of 1545 patients with PV, independent risk factors for overall survival included age 61 years, leukocyte count 10.5 x 10(9)/L, venous thrombosis and abnormal karyotype and for leukemia-free survival age 61 years, leukocyte count 15 x 10(9)/L and abnormal karyotype; median survivals were 23 and 9 years, in the absence or presence of the first two risk factors.

• #3 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes 15 x 10^9/l. […] Median survival for the entire study cohort (n=1545) was projected at 18.9 years and comparison with age- and sex-matched US population suggested a trend towards worse survival (P=0.1). […] However, the particular comparison revealed significantly shortened life expectancy for patients with PV (P<0.001) when the analysis was restricted to one of the seven participating centers (the Mayo Clinic cohort) with the most mature survival data (n=337; 44% of patients followed to death; median survival 14.1 years). [...] Higher leukocyte count, venous thrombosis, leukoerythroblastic blood smear and abnormal karyotype sustained an age-independent adverse prognostic value, whereas thrombocytosis and pruritus were associated with better survival.

• #4

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3768558/

  Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). […] Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P0.001). […] In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0).

• #5 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes 15 x 10^9/l. […] Median survival for the entire study cohort (n=1545) was projected at 18.9 years and comparison with age- and sex-matched US population suggested a trend towards worse survival (P=0.1). […] However, the particular comparison revealed significantly shortened life expectancy for patients with PV (P<0.001) when the analysis was restricted to one of the seven participating centers (the Mayo Clinic cohort) with the most mature survival data (n=337; 44% of patients followed to death; median survival 14.1 years). [...] Higher leukocyte count, venous thrombosis, leukoerythroblastic blood smear and abnormal karyotype sustained an age-independent adverse prognostic value, whereas thrombocytosis and pruritus were associated with better survival.

• #6 Polycythemia Vera: Prognosis and Risk for Progression to Myelofibrosis

  https://www.onclive.com/view/polycythemia-vera-prognosis-and-risk-for-progression-to-myelofibrosis

  The survival of patients with polycythemia vera [PV] is pretty decent compared with those with other myeloid malignancies. Nevertheless, it is slightly decreased compared with age and gender match populations from large database analyses. In one study, the median survival of patients with polycythemia vera was about 14.1 years. In another database analysis, it was about 12.4 years. […] Patients with polycythemia vera can have a number of things happen over the course of the disease. […] Some studies will say as low as 15% of patients over their lifetime will develop myelofibrosis, and some will say as high as 25%. So the truth is probably somewhere in that range. […] Patients with higher JAK2 allele burdens tend to have a higher risk of progression, as well as mutations that might be present in addition to the JAK2 mutation that could derive disease over time into myelofibrosis.

• #7 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. […] Current treatment in PV has not affected the natural history of the disease in regards to overall, leukemia-free or myelofibrosis-free survival, but thrombosis-free survival has been positively affected by treatment with phlebotomy, aspirin and cytoreductive drugs. […] Survival in PV is inferior to that of ET but superior to that of PMF, with estimated medians of 14, 20, and 6 years, respectively; the corresponding figures for patients younger than age 60 years are 24, 33, and 15 years. […] In the largest international study of 1545 patients with PV, independent risk factors for overall survival included age 61 years, leukocyte count 10.5 x 10(9)/L, venous thrombosis and abnormal karyotype and for leukemia-free survival age 61 years, leukocyte count 15 x 10(9)/L and abnormal karyotype; median survivals were 23 and 9 years, in the absence or presence of the first two risk factors.

• #8 Polycythemia Vera Outlook and Life Expectancy

  https://www.healthline.com/health/polycythemia-vera/prognosis-and-life-expectancy

  With treatment, many people with Polycythemia vera (PV) will live at least 14 years after diagnosis. […] The overall median survival period for PV is about 14 years post-diagnosis. […] For people under age 60, the median survival time is about 24 years, which is significantly higher than the overall rate. […] Research shows that the median survival without treatment is 6-18 months, compared to a decade or more with treatment. […] Without treatment, PV can be life threatening. The median survival rate after diagnosis is significantly shorter without treatment compared to with treatment. […] When it does progress despite treatment, it tends to do so slowly. In about a quarter of cases, PV will progress to scarring after about 20 years. About 2-5% of cases transform into leukemia in about 15 years. […] Consistent monitoring and treatment can help improve your life expectancy and quality of life.

• #9 Polycythaemia vera (PV) | MLL

  https://www.mll.com/en/myeloproliferative-neoplasm-mpn/polycythaemia-vera-pv

  Due to strong variation, risk factors affecting survival expectancy should be assessed individually for each patient to the greatest extent possible. Untreated patients with polycythemia vera show an extremely shortened life expectancy (1.5 years) compared to that of treated patients (median survival between 14 and 19 years) (Tefferi Barbui 2019). […] The risk stratification for the therapy decision is based on the risk of thrombosis. […] High JAK2 V617F variant allele frequency (VAF) is also considered a prognostic negative factor for overall survival (Kandua et al. 2023), which also influences thrombosis risk and progression (Zhang et al. 2020, Moliterno et al. 2023, Tefferi Barbui 2023).

• #10 Polycythemia Vera Prognosis – The Patient Story

  https://thepatientstory.com/mpn/polycythemia-vera/prognosis/

  Although no cure exists, the polycythemia vera prognosis is relatively good. Groundbreaking therapies and diligent scientific research have made it possible for patients with polycythemia vera to live on average 20 years after their diagnosis. […] According to Cleveland Clinic, those diagnosed with polycythemia vera have an average life expectancy of someone diagnosed with polycythemia vera is approximately 20 years after diagnosis. However, like any condition, the prognosis varies depending on whether a patient undergoes the recommended treatment plan. […] A study by Ayalew Tefferi and Tiziano Barbui published in the American Journal of Hematology in 2017 shows that life expectancy for those with PV who do not undergo therapy is between 1.5 and 3 years. In comparison, this is extended by 14 years overall with treatment and, on average, an additional 24 years for those under the age of 60.

• #11 Polycythemia Vera Outlook and Life Expectancy

  https://www.healthline.com/health/polycythemia-vera/prognosis-and-life-expectancy

  With treatment, many people with Polycythemia vera (PV) will live at least 14 years after diagnosis. […] The overall median survival period for PV is about 14 years post-diagnosis. […] For people under age 60, the median survival time is about 24 years, which is significantly higher than the overall rate. […] Research shows that the median survival without treatment is 6-18 months, compared to a decade or more with treatment. […] Without treatment, PV can be life threatening. The median survival rate after diagnosis is significantly shorter without treatment compared to with treatment. […] When it does progress despite treatment, it tends to do so slowly. In about a quarter of cases, PV will progress to scarring after about 20 years. About 2-5% of cases transform into leukemia in about 15 years. […] Consistent monitoring and treatment can help improve your life expectancy and quality of life.

• #12

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3768558/

  Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). […] Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P0.001). […] In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0).

• #13 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). […] Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P<0.001). [...] In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0).

• #14 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. […] Current treatment in PV has not affected the natural history of the disease in regards to overall, leukemia-free or myelofibrosis-free survival, but thrombosis-free survival has been positively affected by treatment with phlebotomy, aspirin and cytoreductive drugs. […] Survival in PV is inferior to that of ET but superior to that of PMF, with estimated medians of 14, 20, and 6 years, respectively; the corresponding figures for patients younger than age 60 years are 24, 33, and 15 years. […] In the largest international study of 1545 patients with PV, independent risk factors for overall survival included age 61 years, leukocyte count 10.5 x 10(9)/L, venous thrombosis and abnormal karyotype and for leukemia-free survival age 61 years, leukocyte count 15 x 10(9)/L and abnormal karyotype; median survivals were 23 and 9 years, in the absence or presence of the first two risk factors.

• #15 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. […] Current treatment in PV has not affected the natural history of the disease in regards to overall, leukemia-free or myelofibrosis-free survival, but thrombosis-free survival has been positively affected by treatment with phlebotomy, aspirin and cytoreductive drugs. […] Survival in PV is inferior to that of ET but superior to that of PMF, with estimated medians of 14, 20, and 6 years, respectively; the corresponding figures for patients younger than age 60 years are 24, 33, and 15 years. […] In the largest international study of 1545 patients with PV, independent risk factors for overall survival included age 61 years, leukocyte count 10.5 x 10(9)/L, venous thrombosis and abnormal karyotype and for leukemia-free survival age 61 years, leukocyte count 15 x 10(9)/L and abnormal karyotype; median survivals were 23 and 9 years, in the absence or presence of the first two risk factors.

• #16 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes 15 x 10^9/l. […] Median survival for the entire study cohort (n=1545) was projected at 18.9 years and comparison with age- and sex-matched US population suggested a trend towards worse survival (P=0.1). […] However, the particular comparison revealed significantly shortened life expectancy for patients with PV (P<0.001) when the analysis was restricted to one of the seven participating centers (the Mayo Clinic cohort) with the most mature survival data (n=337; 44% of patients followed to death; median survival 14.1 years). [...] Higher leukocyte count, venous thrombosis, leukoerythroblastic blood smear and abnormal karyotype sustained an age-independent adverse prognostic value, whereas thrombocytosis and pruritus were associated with better survival.

• #17 Polycythaemia vera (PV) | MLL

  https://www.mll.com/en/myeloproliferative-neoplasm-mpn/polycythaemia-vera-pv

  Due to strong variation, risk factors affecting survival expectancy should be assessed individually for each patient to the greatest extent possible. Untreated patients with polycythemia vera show an extremely shortened life expectancy (1.5 years) compared to that of treated patients (median survival between 14 and 19 years) (Tefferi Barbui 2019). […] The risk stratification for the therapy decision is based on the risk of thrombosis. […] High JAK2 V617F variant allele frequency (VAF) is also considered a prognostic negative factor for overall survival (Kandua et al. 2023), which also influences thrombosis risk and progression (Zhang et al. 2020, Moliterno et al. 2023, Tefferi Barbui 2023).

• #18 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  The prognostic relevance of karyotype in PV was recently confirmed by subsequent Mayo Clinic and MD Anderson Cancer Center (MDACC) reports; in both studies, abnormal karyotype was reported in approximately 20% of patients and adversely affected overall and transformation-free survival. […] Some of these mutations, in particular ASXL1, SRSF2, or IDH2, have been shown to adversely impact overall and transformation-free survival; median survival of patients with and without adverse mutations was 7.7 vs. 16.9 years, respectively. […] The major life-threatening complications in PV are leukemic transformation, fibrotic progression and thrombosis, with incidence ranges of 5.5-18.7%, 6-14%, and 6-17%, respectively, over the course of 15, 15, and 3 years. […] These observations were taken into consideration in formulating our treatment recommendations for high-risk disease.

• #19 Systemic Inflammatory Index in Polycythemia Vera and Its Prognostic Implications

  https://www.mdpi.com/2077-0383/13/15/4459

  The systemic inflammatory index (SII) has demonstrated an association with all-cause and cardiovascular mortality in the general population and it could be prognostically useful in PV, as it integrates all of the aforementioned MPN features through different CBC components (ANC, platelet count, and ALC). […] The aim of this study was to investigate the SII in PV patients, its clinical correlations, and the potential prognostic impact of the SII on thrombotic risk and survival. […] The presented results complement prior studies evaluating the prognostic performance of different CBC components for thrombotic and survival risk predictions and offer more options to personalize PV treatments. […] In patients with PV, a higher SII may be associated with an inferior TTT and OS; the association of a high SII with thrombotic risk outperformed the ELN score and was independently associated with an inferior OS, whereas higher SII, the AAA risk model, and the CCI independently of each other predicted thrombotic events. Conversely, the AAA risk score outperformed the high SII with respect to OS prediction. Higher SII was also able to further refine the ELN high-risk patient subgroup for both thrombotic and survival risks, especially in individuals over 60 years of age. Finally, even though all evaluated CBC indices demonstrated low to modest prognostic properties, the SII outperformed other evaluated parameters (WBC, ANC, ALC, platelet count, NLR, and PLR) with respect to TTT and OS predictions.

• #20 Metabolic Biomarkers Affecting Cell Proliferation and Prognosis in Polycythemia Vera

  https://www.mdpi.com/2072-6694/14/19/4913

  Metabolic Biomarkers Affecting Cell Proliferation and Prognosis in Polycythemia Vera […] Polycythemia vera (PV) is a malignant neoplastic disease. […] Our study described the global metabolic profile of PV patients, analyzed their relationship with cell proliferation, screened prognosis-related metabolic biomarkers. […] The elevated levels of four potential biomarkers may provide a reference for poor prognosis in PV patients. […] A set of potential prognostic metabolic biomarkers containing four metabolites was identified by a receiver operating characteristic (ROC) curve according to the risk stratification of the PV patients and their combined AUC value of 0.952, with a sensitivity of 90.905% and specificity of 90.909% at the optimal cutoff point. […] In this study, we found that the levels of Cer(d18:2/22:6-2OH(7S, 17S)) and SM(d18:0/PGF1α) in the JAK2-mutated PV patients all increased significantly and showed a significant correlation with the JAK2 mutational burden and peripheral blood cell counts, suggesting that these two metabolites may promote the proliferation of peripheral blood cells in PV patients under the regulation of the JAK-STAT pathway. […] Elevated levels of Cer(d18:2/22:6-2OH(7S, 17S)), SM(d18:0/PGF1α), CerP(d18:1/16:0), and octadec-13-enoylcarnitine could be used as potential metabolic biomarkers of poor prognosis in patients with PV.

• #21 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes 15 x 10^9/l. […] Median survival for the entire study cohort (n=1545) was projected at 18.9 years and comparison with age- and sex-matched US population suggested a trend towards worse survival (P=0.1). […] However, the particular comparison revealed significantly shortened life expectancy for patients with PV (P<0.001) when the analysis was restricted to one of the seven participating centers (the Mayo Clinic cohort) with the most mature survival data (n=337; 44% of patients followed to death; median survival 14.1 years). [...] Higher leukocyte count, venous thrombosis, leukoerythroblastic blood smear and abnormal karyotype sustained an age-independent adverse prognostic value, whereas thrombocytosis and pruritus were associated with better survival.

• #22

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3768558/

  Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). […] Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P0.001). […] In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0).

• #23 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). […] Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P<0.001). [...] In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0).

• #24 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  A total of 50 (3%) cases of post-PV AML were documented and occurred at a median of 10.8 years (range 0.5-22.3) from diagnosis. […] Both univariate and multivariable analyses of parameters at diagnosis identified older age (HR, 6.3; 95% CI: 1.8-22.0), abnormal karyotype (HR, 3.9; 95% CI: 1.2-13.1) and leukocyte count 15 x 10^9/l (HR, 3.9; 95% CI: 1.3-11.6) as independent risk factors for leukemia-free survival. […] The new prognostic model applied to the training set (the Mayo Clinic cohort) displayed excellent discrimination between high-risk (n=155; median survival=8.3 years; HR, 11.1; 95% CI: 6.3-19.6), intermediate-risk (n=100; median survival=15 years; HR, 3.5; 95% CI: 1.9-6.2) and low-risk (n=82; median survival=26 years) patient groups and was subsequently validated in the entire study cohort (n=1545).

• #25 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  A total of 50 (3%) cases of post-PV AML were documented and occurred at a median of 10.8 years (range 0.5-22.3) from diagnosis. […] Both univariate and multivariable analyses of parameters at diagnosis identified older age (HR, 6.3; 95% CI: 1.8-22.0), abnormal karyotype (HR, 3.9; 95% CI: 1.2-13.1) and leukocyte count 15 x 10^9/l (HR, 3.9; 95% CI: 1.3-11.6) as independent risk factors for leukemia-free survival. […] The new prognostic model applied to the training set (the Mayo Clinic cohort) displayed excellent discrimination between high-risk (n=155; median survival=8.3 years; HR, 11.1; 95% CI: 6.3-19.6), intermediate-risk (n=100; median survival=15 years; HR, 3.5; 95% CI: 1.9-6.2) and low-risk (n=82; median survival=26 years) patient groups and was subsequently validated in the entire study cohort (n=1545).

• #26 Polycythemia Vera – StatPearls – NCBI Bookshelf

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

  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. […] Polycythemia vera-related complications and mortality are related to thrombosis, hemorrhage, peptic ulcer disease, myelofibrosis, acute leukemia, or myelodysplastic syndrome (MDS).

• #27 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  The prognostic relevance of karyotype in PV was recently confirmed by subsequent Mayo Clinic and MD Anderson Cancer Center (MDACC) reports; in both studies, abnormal karyotype was reported in approximately 20% of patients and adversely affected overall and transformation-free survival. […] Some of these mutations, in particular ASXL1, SRSF2, or IDH2, have been shown to adversely impact overall and transformation-free survival; median survival of patients with and without adverse mutations was 7.7 vs. 16.9 years, respectively. […] The major life-threatening complications in PV are leukemic transformation, fibrotic progression and thrombosis, with incidence ranges of 5.5-18.7%, 6-14%, and 6-17%, respectively, over the course of 15, 15, and 3 years. […] These observations were taken into consideration in formulating our treatment recommendations for high-risk disease.

• #28 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes 15 x 10^9/l. […] Median survival for the entire study cohort (n=1545) was projected at 18.9 years and comparison with age- and sex-matched US population suggested a trend towards worse survival (P=0.1). […] However, the particular comparison revealed significantly shortened life expectancy for patients with PV (P<0.001) when the analysis was restricted to one of the seven participating centers (the Mayo Clinic cohort) with the most mature survival data (n=337; 44% of patients followed to death; median survival 14.1 years). [...] Higher leukocyte count, venous thrombosis, leukoerythroblastic blood smear and abnormal karyotype sustained an age-independent adverse prognostic value, whereas thrombocytosis and pruritus were associated with better survival.

• #29 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  A total of 50 (3%) cases of post-PV AML were documented and occurred at a median of 10.8 years (range 0.5-22.3) from diagnosis. […] Both univariate and multivariable analyses of parameters at diagnosis identified older age (HR, 6.3; 95% CI: 1.8-22.0), abnormal karyotype (HR, 3.9; 95% CI: 1.2-13.1) and leukocyte count 15 x 10^9/l (HR, 3.9; 95% CI: 1.3-11.6) as independent risk factors for leukemia-free survival. […] The new prognostic model applied to the training set (the Mayo Clinic cohort) displayed excellent discrimination between high-risk (n=155; median survival=8.3 years; HR, 11.1; 95% CI: 6.3-19.6), intermediate-risk (n=100; median survival=15 years; HR, 3.5; 95% CI: 1.9-6.2) and low-risk (n=82; median survival=26 years) patient groups and was subsequently validated in the entire study cohort (n=1545).

• #30

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3768558/

  The inclusion of cytogenetic information to the multivariable model reduced the number of fully informative cases from 964 to 383 and resulted in the loss of significance attached to thrombocytosis (P=0.09) and leukoerythroblastosis (P=0.36; Table 3). […] A total of 50 (3%) cases of post-PV AML were documented and occurred at a median of 10.8 years (range 0.5-22.3) from diagnosis. […] Both univariate and multivariable analyses of parameters at diagnosis identified older age (HR, 6.3; 95% CI: 1.8-22.0), abnormal karyotype (HR, 3.9; 95% CI: 1.2-13.1) and leukocyte count 15 10^9/l (HR, 3.9; 95% CI: 1.3-11.6) as independent risk factors for leukemia-free survival. […] The adverse survival impact of otherwise unspecified thrombosis’ have previously been reported in both ET and PV, and based on the results from this study, it would be important to further clarify if the specific association involves arterial or venous thrombosis.

• #31 Polycythemia Vera Outlook and Life Expectancy

  https://www.healthline.com/health/polycythemia-vera/prognosis-and-life-expectancy

  With treatment, many people with Polycythemia vera (PV) will live at least 14 years after diagnosis. […] The overall median survival period for PV is about 14 years post-diagnosis. […] For people under age 60, the median survival time is about 24 years, which is significantly higher than the overall rate. […] Research shows that the median survival without treatment is 6-18 months, compared to a decade or more with treatment. […] Without treatment, PV can be life threatening. The median survival rate after diagnosis is significantly shorter without treatment compared to with treatment. […] When it does progress despite treatment, it tends to do so slowly. In about a quarter of cases, PV will progress to scarring after about 20 years. About 2-5% of cases transform into leukemia in about 15 years. […] Consistent monitoring and treatment can help improve your life expectancy and quality of life.

• #32 Polycythemia Vera Prognosis – The Patient Story

  https://thepatientstory.com/mpn/polycythemia-vera/prognosis/

  The prognosis for polycythemia vera improves with exercise and a healthy diet. However, complications associated with PV can complicate the accuracy of a prognosis. […] A polycythemia vera prognosis can change based on the progression of the disease. Over time a patient with PV could develop blood clots or even more severe forms of cancer. […] While it is possible for polycythemia vera to progress, only about 3% of PV cases progress fully to Acute Myeloid Leukemia. […] It’s important to note that the progression of polycythemia vera is only a possibility and not a certainty. The disease may never progress into further stages with proper polycythemia vera treatment and therapy.

• #33

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3768558/

  The inclusion of cytogenetic information to the multivariable model reduced the number of fully informative cases from 964 to 383 and resulted in the loss of significance attached to thrombocytosis (P=0.09) and leukoerythroblastosis (P=0.36; Table 3). […] A total of 50 (3%) cases of post-PV AML were documented and occurred at a median of 10.8 years (range 0.5-22.3) from diagnosis. […] Both univariate and multivariable analyses of parameters at diagnosis identified older age (HR, 6.3; 95% CI: 1.8-22.0), abnormal karyotype (HR, 3.9; 95% CI: 1.2-13.1) and leukocyte count 15 10^9/l (HR, 3.9; 95% CI: 1.3-11.6) as independent risk factors for leukemia-free survival. […] The adverse survival impact of otherwise unspecified thrombosis’ have previously been reported in both ET and PV, and based on the results from this study, it would be important to further clarify if the specific association involves arterial or venous thrombosis.

• #34 Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study | Leukemia

  https://www.nature.com/articles/leu2013163

  A total of 50 (3%) cases of post-PV AML were documented and occurred at a median of 10.8 years (range 0.5-22.3) from diagnosis. […] Both univariate and multivariable analyses of parameters at diagnosis identified older age (HR, 6.3; 95% CI: 1.8-22.0), abnormal karyotype (HR, 3.9; 95% CI: 1.2-13.1) and leukocyte count 15 x 10^9/l (HR, 3.9; 95% CI: 1.3-11.6) as independent risk factors for leukemia-free survival. […] The new prognostic model applied to the training set (the Mayo Clinic cohort) displayed excellent discrimination between high-risk (n=155; median survival=8.3 years; HR, 11.1; 95% CI: 6.3-19.6), intermediate-risk (n=100; median survival=15 years; HR, 3.5; 95% CI: 1.9-6.2) and low-risk (n=82; median survival=26 years) patient groups and was subsequently validated in the entire study cohort (n=1545).

• #35 Polycythemia Vera: Prognosis and Risk for Progression to Myelofibrosis

  https://www.onclive.com/view/polycythemia-vera-prognosis-and-risk-for-progression-to-myelofibrosis

  The survival of patients with polycythemia vera [PV] is pretty decent compared with those with other myeloid malignancies. Nevertheless, it is slightly decreased compared with age and gender match populations from large database analyses. In one study, the median survival of patients with polycythemia vera was about 14.1 years. In another database analysis, it was about 12.4 years. […] Patients with polycythemia vera can have a number of things happen over the course of the disease. […] Some studies will say as low as 15% of patients over their lifetime will develop myelofibrosis, and some will say as high as 25%. So the truth is probably somewhere in that range. […] Patients with higher JAK2 allele burdens tend to have a higher risk of progression, as well as mutations that might be present in addition to the JAK2 mutation that could derive disease over time into myelofibrosis.

• #36 Polycythemia Vera Outlook and Life Expectancy

  https://www.healthline.com/health/polycythemia-vera/prognosis-and-life-expectancy

  With treatment, many people with Polycythemia vera (PV) will live at least 14 years after diagnosis. […] The overall median survival period for PV is about 14 years post-diagnosis. […] For people under age 60, the median survival time is about 24 years, which is significantly higher than the overall rate. […] Research shows that the median survival without treatment is 6-18 months, compared to a decade or more with treatment. […] Without treatment, PV can be life threatening. The median survival rate after diagnosis is significantly shorter without treatment compared to with treatment. […] When it does progress despite treatment, it tends to do so slowly. In about a quarter of cases, PV will progress to scarring after about 20 years. About 2-5% of cases transform into leukemia in about 15 years. […] Consistent monitoring and treatment can help improve your life expectancy and quality of life.

• #37 Polycythemia Vera: Prognosis and Risk for Progression to Myelofibrosis

  https://www.onclive.com/view/polycythemia-vera-prognosis-and-risk-for-progression-to-myelofibrosis

  The survival of patients with polycythemia vera [PV] is pretty decent compared with those with other myeloid malignancies. Nevertheless, it is slightly decreased compared with age and gender match populations from large database analyses. In one study, the median survival of patients with polycythemia vera was about 14.1 years. In another database analysis, it was about 12.4 years. […] Patients with polycythemia vera can have a number of things happen over the course of the disease. […] Some studies will say as low as 15% of patients over their lifetime will develop myelofibrosis, and some will say as high as 25%. So the truth is probably somewhere in that range. […] Patients with higher JAK2 allele burdens tend to have a higher risk of progression, as well as mutations that might be present in addition to the JAK2 mutation that could derive disease over time into myelofibrosis.

• #38 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  The prognostic relevance of karyotype in PV was recently confirmed by subsequent Mayo Clinic and MD Anderson Cancer Center (MDACC) reports; in both studies, abnormal karyotype was reported in approximately 20% of patients and adversely affected overall and transformation-free survival. […] Some of these mutations, in particular ASXL1, SRSF2, or IDH2, have been shown to adversely impact overall and transformation-free survival; median survival of patients with and without adverse mutations was 7.7 vs. 16.9 years, respectively. […] The major life-threatening complications in PV are leukemic transformation, fibrotic progression and thrombosis, with incidence ranges of 5.5-18.7%, 6-14%, and 6-17%, respectively, over the course of 15, 15, and 3 years. […] These observations were taken into consideration in formulating our treatment recommendations for high-risk disease.

• #39 Polycythemia Vera: Prognosis and Risk for Progression to Myelofibrosis

  https://www.onclive.com/view/polycythemia-vera-prognosis-and-risk-for-progression-to-myelofibrosis

  There is clearly a risk of progression in one’s lifetime with polycythemia vera for getting myelofibrosis. […] We know that if you’re older than 60 years or you had a prior blood clot you have a higher chance for blood clots in general. […] There are scoring systems such as the MYSEC-PM, for example, that aim to specially focus on patients with post-ET and post-PV myelofibrosis.

• #40 Systemic Inflammatory Index in Polycythemia Vera and Its Prognostic Implications

  https://www.mdpi.com/2077-0383/13/15/4459

  The systemic inflammatory index (SII) has demonstrated an association with all-cause and cardiovascular mortality in the general population and it could be prognostically useful in PV, as it integrates all of the aforementioned MPN features through different CBC components (ANC, platelet count, and ALC). […] The aim of this study was to investigate the SII in PV patients, its clinical correlations, and the potential prognostic impact of the SII on thrombotic risk and survival. […] The presented results complement prior studies evaluating the prognostic performance of different CBC components for thrombotic and survival risk predictions and offer more options to personalize PV treatments. […] In patients with PV, a higher SII may be associated with an inferior TTT and OS; the association of a high SII with thrombotic risk outperformed the ELN score and was independently associated with an inferior OS, whereas higher SII, the AAA risk model, and the CCI independently of each other predicted thrombotic events. Conversely, the AAA risk score outperformed the high SII with respect to OS prediction. Higher SII was also able to further refine the ELN high-risk patient subgroup for both thrombotic and survival risks, especially in individuals over 60 years of age. Finally, even though all evaluated CBC indices demonstrated low to modest prognostic properties, the SII outperformed other evaluated parameters (WBC, ANC, ALC, platelet count, NLR, and PLR) with respect to TTT and OS predictions.

• #41 Polycythemia vera treatment algorithm 2018 | Blood Cancer Journal

  https://www.nature.com/articles/s41408-017-0042-7

  Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. […] Current treatment in PV has not affected the natural history of the disease in regards to overall, leukemia-free or myelofibrosis-free survival, but thrombosis-free survival has been positively affected by treatment with phlebotomy, aspirin and cytoreductive drugs. […] Survival in PV is inferior to that of ET but superior to that of PMF, with estimated medians of 14, 20, and 6 years, respectively; the corresponding figures for patients younger than age 60 years are 24, 33, and 15 years. […] In the largest international study of 1545 patients with PV, independent risk factors for overall survival included age 61 years, leukocyte count 10.5 x 10(9)/L, venous thrombosis and abnormal karyotype and for leukemia-free survival age 61 years, leukocyte count 15 x 10(9)/L and abnormal karyotype; median survivals were 23 and 9 years, in the absence or presence of the first two risk factors.

• #42 Polycythemia Vera Outlook and Life Expectancy

  https://www.healthline.com/health/polycythemia-vera/prognosis-and-life-expectancy

  With treatment, many people with Polycythemia vera (PV) will live at least 14 years after diagnosis. […] The overall median survival period for PV is about 14 years post-diagnosis. […] For people under age 60, the median survival time is about 24 years, which is significantly higher than the overall rate. […] Research shows that the median survival without treatment is 6-18 months, compared to a decade or more with treatment. […] Without treatment, PV can be life threatening. The median survival rate after diagnosis is significantly shorter without treatment compared to with treatment. […] When it does progress despite treatment, it tends to do so slowly. In about a quarter of cases, PV will progress to scarring after about 20 years. About 2-5% of cases transform into leukemia in about 15 years. […] Consistent monitoring and treatment can help improve your life expectancy and quality of life.

• #43 Polycythemia vera (PRV) any other young people? – Living with and after blood cancer – Blood Cancer UK Online Community Forum

  https://forum.bloodcancer.org.uk/t/polycythemia-vera-prv-any-other-young-people/10821

  There is a lot of misinformation out there so its best to ensure you have confidence in the source. […] I cant offer a prognosis but there are so many folk whom were diagnosed at a similar age are still going strong in their 70s-80s. […] Its important to note that it seems on recently that they are getting better at testing for Polycythaemia vera (PV) and in the past it was more common people were being diagnosed in their late 60s early 70s when they presented common symptoms or after a series of tests for other conditions. This would explain the prognosis of 10-20 years. […] A common aphorism about Polycythaemia vera (PV) is that we are much more likely to die with it than from it. […] Those Polycythaemia vera (PV) prognoses we can find online of 10-20 years are because people were often diagnosed in older age and their life expectancies at those ages were already about the same. Current research shows we can live long healthy lives into old age.

• #44 Polycythaemia vera (PV) | MLL

  https://www.mll.com/en/myeloproliferative-neoplasm-mpn/polycythaemia-vera-pv

  Due to strong variation, risk factors affecting survival expectancy should be assessed individually for each patient to the greatest extent possible. Untreated patients with polycythemia vera show an extremely shortened life expectancy (1.5 years) compared to that of treated patients (median survival between 14 and 19 years) (Tefferi Barbui 2019). […] The risk stratification for the therapy decision is based on the risk of thrombosis. […] High JAK2 V617F variant allele frequency (VAF) is also considered a prognostic negative factor for overall survival (Kandua et al. 2023), which also influences thrombosis risk and progression (Zhang et al. 2020, Moliterno et al. 2023, Tefferi Barbui 2023).

• #45 Prognosis for polycythaemia vera (PV) | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/polycythaemia-vera-pv/pv-prognosis/

  Your prognosis (what will happen in the future) depends on things that are individual to you, such as your age and fitness, whether you have had blood clots in the past, and which genetic mutation you have. Your specialist doctor (haematologist) is the best person to ask about your prognosis because they have the best understanding of your overall health and the type of the PV you have. […] The risk of either of these things happening is low for everyone, but there is some evidence that it increases the longer you have PV. If you have been diagnosed with PV at a younger age, this is understandably worrying. […] Talk to your hospital team if you are concerned about this or anything else about your prognosis they may be able to reassure you or refer you for psychological help.

• #46 Prognosis for polycythaemia vera (PV) | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/polycythaemia-vera-pv/pv-prognosis/

  Your prognosis (what will happen in the future) depends on things that are individual to you, such as your age and fitness, whether you have had blood clots in the past, and which genetic mutation you have. Your specialist doctor (haematologist) is the best person to ask about your prognosis because they have the best understanding of your overall health and the type of the PV you have. […] The risk of either of these things happening is low for everyone, but there is some evidence that it increases the longer you have PV. If you have been diagnosed with PV at a younger age, this is understandably worrying. […] Talk to your hospital team if you are concerned about this or anything else about your prognosis they may be able to reassure you or refer you for psychological help.

• #47 Polycythemia vera (PRV) any other young people? – Living with and after blood cancer – Blood Cancer UK Online Community Forum

  https://forum.bloodcancer.org.uk/t/polycythemia-vera-prv-any-other-young-people/10821

  There is a lot of misinformation out there so its best to ensure you have confidence in the source. […] I cant offer a prognosis but there are so many folk whom were diagnosed at a similar age are still going strong in their 70s-80s. […] Its important to note that it seems on recently that they are getting better at testing for Polycythaemia vera (PV) and in the past it was more common people were being diagnosed in their late 60s early 70s when they presented common symptoms or after a series of tests for other conditions. This would explain the prognosis of 10-20 years. […] A common aphorism about Polycythaemia vera (PV) is that we are much more likely to die with it than from it. […] Those Polycythaemia vera (PV) prognoses we can find online of 10-20 years are because people were often diagnosed in older age and their life expectancies at those ages were already about the same. Current research shows we can live long healthy lives into old age.

• #48 Metabolic Biomarkers Affecting Cell Proliferation and Prognosis in Polycythemia Vera

  https://www.mdpi.com/2072-6694/14/19/4913

  Metabolic Biomarkers Affecting Cell Proliferation and Prognosis in Polycythemia Vera […] Polycythemia vera (PV) is a malignant neoplastic disease. […] Our study described the global metabolic profile of PV patients, analyzed their relationship with cell proliferation, screened prognosis-related metabolic biomarkers. […] The elevated levels of four potential biomarkers may provide a reference for poor prognosis in PV patients. […] A set of potential prognostic metabolic biomarkers containing four metabolites was identified by a receiver operating characteristic (ROC) curve according to the risk stratification of the PV patients and their combined AUC value of 0.952, with a sensitivity of 90.905% and specificity of 90.909% at the optimal cutoff point. […] In this study, we found that the levels of Cer(d18:2/22:6-2OH(7S, 17S)) and SM(d18:0/PGF1α) in the JAK2-mutated PV patients all increased significantly and showed a significant correlation with the JAK2 mutational burden and peripheral blood cell counts, suggesting that these two metabolites may promote the proliferation of peripheral blood cells in PV patients under the regulation of the JAK-STAT pathway. […] Elevated levels of Cer(d18:2/22:6-2OH(7S, 17S)), SM(d18:0/PGF1α), CerP(d18:1/16:0), and octadec-13-enoylcarnitine could be used as potential metabolic biomarkers of poor prognosis in patients with PV.

• #49 Systemic Inflammatory Index in Polycythemia Vera and Its Prognostic Implications

  https://www.mdpi.com/2077-0383/13/15/4459

  The systemic inflammatory index (SII) has demonstrated an association with all-cause and cardiovascular mortality in the general population and it could be prognostically useful in PV, as it integrates all of the aforementioned MPN features through different CBC components (ANC, platelet count, and ALC). […] The aim of this study was to investigate the SII in PV patients, its clinical correlations, and the potential prognostic impact of the SII on thrombotic risk and survival. […] The presented results complement prior studies evaluating the prognostic performance of different CBC components for thrombotic and survival risk predictions and offer more options to personalize PV treatments. […] In patients with PV, a higher SII may be associated with an inferior TTT and OS; the association of a high SII with thrombotic risk outperformed the ELN score and was independently associated with an inferior OS, whereas higher SII, the AAA risk model, and the CCI independently of each other predicted thrombotic events. Conversely, the AAA risk score outperformed the high SII with respect to OS prediction. Higher SII was also able to further refine the ELN high-risk patient subgroup for both thrombotic and survival risks, especially in individuals over 60 years of age. Finally, even though all evaluated CBC indices demonstrated low to modest prognostic properties, the SII outperformed other evaluated parameters (WBC, ANC, ALC, platelet count, NLR, and PLR) with respect to TTT and OS predictions.

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