Materiały źródłowe

• #1

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  Myelofibrosis (MF) includes prefibrotic primary MF (pre-PMF), overt-PMF and secondary MF (SMF). Median overall survival (OS) of pre-PMF, overt-PMF and SMF patients is around 14 years, seven and nine years, respectively. […] Discoveries on the impact of the biological architecture on OS have led to the design of integrated scores to predict survival in PMF. […] Prognostic models guide prediction of OS and treatment planning in MF, therefore, their timely application is critical in the personalized approach of MF patients. […] Patients affected by MF have a significantly reduced outcome, with a median OS of 14 years, seven and nine years in pre-PMF, overt-PMF and SMF, respectively. […] Nonetheless, in recent years improvement in prognosis has been registered. […] The MYSEC cohort, that represents to date the largest dataset of SMF patients, allowed to generate a specific clinical-molecular prognostic score, the MYSEC-Prognostic Model (MYSEC-PM).

• #2 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Among the myeloproliferative diseases, myelofibrosis is a widely heterogeneous entity characterized by a highly variable prognosis. […] Identifying who deserves more invasive treatments, such as bone marrow transplantation, is a critical clinical need. […] In fact, overall survival can range from fewer than two years to a life expectancy comparable to the general population and greater than 20 years. […] For this purpose, numerous prognostic scores have been proposed over the years and used to help physicians estimate median overall survival (mOS) and leukemia-free survival (LFS). […] The objective of this review is to collect all the currently available prognostic scores to explain in a clear, concise, and easily usable way which could be the most suitable model to apply at any time during patient follow-up, dwelling on the most recent proposals to have seen the light and that will probably be used shortly.

• #3 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Among the myeloproliferative diseases, myelofibrosis is a widely heterogeneous entity characterized by a highly variable prognosis. […] Identifying who deserves more invasive treatments, such as bone marrow transplantation, is a critical clinical need. […] In fact, overall survival can range from fewer than two years to a life expectancy comparable to the general population and greater than 20 years. […] For this purpose, numerous prognostic scores have been proposed over the years and used to help physicians estimate median overall survival (mOS) and leukemia-free survival (LFS). […] The objective of this review is to collect all the currently available prognostic scores to explain in a clear, concise, and easily usable way which could be the most suitable model to apply at any time during patient follow-up, dwelling on the most recent proposals to have seen the light and that will probably be used shortly.

• #4 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Among the myeloproliferative diseases, myelofibrosis is a widely heterogeneous entity characterized by a highly variable prognosis. […] Identifying who deserves more invasive treatments, such as bone marrow transplantation, is a critical clinical need. […] In fact, overall survival can range from fewer than two years to a life expectancy comparable to the general population and greater than 20 years. […] For this purpose, numerous prognostic scores have been proposed over the years and used to help physicians estimate median overall survival (mOS) and leukemia-free survival (LFS). […] The objective of this review is to collect all the currently available prognostic scores to explain in a clear, concise, and easily usable way which could be the most suitable model to apply at any time during patient follow-up, dwelling on the most recent proposals to have seen the light and that will probably be used shortly.

• #5 Myelofibrosis prognosis: Life expectancy, treatment options & more

  https://www.medicalnewstoday.com/articles/myelofibrosis-prognosis

  The outlook for someone with myelofibrosis depends on factors such as age, blood cell counts, and symptoms. For some people, the condition progresses more rapidly than others. […] Therefore, the MF outlook varies significantly. […] Predicting the life expectancy of someone with MF is challenging. […] The approximate life expectancy for those with MF is as follows: 15.4 years for low risk individuals, 6.5 years for intermediate-1 (INT-1) risk individuals, 2.9 years for INT-2 risk individuals, 1.3 years for high risk individuals. […] However, while outcome data can illustrate how people with MF have fared in response to treatments, it may not precisely predict how an individual will respond. […] A person’s myelofibrosis prognosis depends on various factors, and some people remain symptom-free for many years. […] However, for those with risk factors such as being over 65 years old, having whole-body symptoms or anemia, and having specific genetic abnormalities, the outlook may be less favorable.

• #6 Myelofibrosis: Prognosis and Life Expectancy

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

  Life expectancy for myelofibrosis varies based on age, blood cell counts, and symptoms. Some experience rapid progression, while others may live longer without symptoms. Treatment can help extend your life span. […] Predicting the outlook for MF is difficult and depends on many factors. […] However, doctors and researchers have identified some factors that can help predict a persons outlook with MF. These factors are used in the International Prognosis Scoring System (IPSS) to help doctors predict average years of survival. […] Its important to note that these survival estimates are based on survival averages and currently available treatments. As newer treatments are developed, survival rates may also change. […] Meeting one of the factors below means the average survival rate is about six years. Meeting three or more can lower the expected survival rate to around one and almost three years.

• #7 How Long Will I Live With Myelofibrosis? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/articles/understanding-mf-prognosis

  Around 10-20% of people with myelofibrosis will progress to acute myeloid leukemia. This type of leukemia is challenging to treat and has a significant impact on a persons life. For this reason, myelofibrosis prognosis will be directly linked to the patients risk of progressing to leukemia. […] A helpful tool for doctors to assess risk is the DIPSS dynamic international prognostic scoring system, which takes into account the following factors: Age (65 or older), Symptoms (non-specific indicators like fever, fatigue, appetite loss, etc), Anemia, High white blood cell count (leukocytosis), Circulating immature blood cells (blasts), Need for transfusions, Genetic mutations in the bone marrow cells. […] These risk factors help quickly assess a patient to calculate an approximate life expectancy. However, doctors consider other factors when meeting patients and learning more about their lifestyle, habits, family history, and overall health.

• #8 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  Myelofibrosis is a type of myeloproliferative neoplasm (MPN) with a median survival of 6 years. A median is a midpoint, which means that some people live less than six years, and about the same number of people can live longer than six years. Overall, fewer than 20% of patients with myelofibrosis are predicted to survive longer than 10 years. However, current advancements in myelofibrosis treatment are improving patient prognosis. […] The prognosis for myelofibrosis varies widely depending on several factors: […] Patients with early-stage disease generally have a better prognosis than those with advanced-stage disease. […] Age can impact prognosis, with older patients potentially facing more complications and challenges in managing the disease. […] Prognosis is influenced by the severity of symptoms and complications, such as an enlarged spleen or recurrent infections.

• #9 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  Specific genetic mutations, such as mutations in genes like JAK2, CALR, or MPL, can significantly impact prognosis by affecting disease progression and treatment response. […] Advances in treatment options, such as targeted therapies and bone marrow transplantation, have led to better outcomes for many people with myelofibrosis. However, individual responses to treatment can vary, and how well a person responds to and tolerates the treatment can greatly impact their prognosis. […] In some cases, myelofibrosis can transform into acute leukemia, which typically has a poorer prognosis. […] Older age correlates with shorter survival in patients with myelofibrosis. […] Patients diagnosed with intermediate or high-risk primary myelofibrosis will have a poorer prognosis (usually less than 5 years survival), regardless of age.

• #10 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  Specific genetic mutations, such as mutations in genes like JAK2, CALR, or MPL, can significantly impact prognosis by affecting disease progression and treatment response. […] Advances in treatment options, such as targeted therapies and bone marrow transplantation, have led to better outcomes for many people with myelofibrosis. However, individual responses to treatment can vary, and how well a person responds to and tolerates the treatment can greatly impact their prognosis. […] In some cases, myelofibrosis can transform into acute leukemia, which typically has a poorer prognosis. […] Older age correlates with shorter survival in patients with myelofibrosis. […] Patients diagnosed with intermediate or high-risk primary myelofibrosis will have a poorer prognosis (usually less than 5 years survival), regardless of age.

• #11 Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements

  https://www.mdpi.com/2072-6694/16/3/514

  Myelofibrosis (MF) is an essential element of primary myelofibrosis, whereas secondary MF may develop in the advanced stages of other myeloid neoplasms, especially polycythemia vera and essential thrombocythemia. Over the last two decades, advances in molecular diagnostic techniques, particularly the integration of next-generation sequencing in clinical laboratories, have revolutionized the diagnosis, classification, and clinical decision making of myelofibrosis. […] Prognostic prediction plays a pivotal role in guiding the treatment of myelofibrosis. Mutation profiles and cytogenetic abnormalities have been integrated into advanced prognostic scoring systems and personalized risk stratification for MF. […] The evolving landscape of prognostic systems for myelofibrosis underscores the increasing recognition of complex clinical, cytogenetic, and molecular factors for predicting disease outcomes. The prognostic value of HMR mutations in MIPSS70+ V2.0 has been confirmed by recent clinical study results.

• #12 Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements

  https://www.mdpi.com/2072-6694/16/3/514

  Myelofibrosis (MF) is an essential element of primary myelofibrosis, whereas secondary MF may develop in the advanced stages of other myeloid neoplasms, especially polycythemia vera and essential thrombocythemia. Over the last two decades, advances in molecular diagnostic techniques, particularly the integration of next-generation sequencing in clinical laboratories, have revolutionized the diagnosis, classification, and clinical decision making of myelofibrosis. […] Prognostic prediction plays a pivotal role in guiding the treatment of myelofibrosis. Mutation profiles and cytogenetic abnormalities have been integrated into advanced prognostic scoring systems and personalized risk stratification for MF. […] The evolving landscape of prognostic systems for myelofibrosis underscores the increasing recognition of complex clinical, cytogenetic, and molecular factors for predicting disease outcomes. The prognostic value of HMR mutations in MIPSS70+ V2.0 has been confirmed by recent clinical study results.

• #13 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Two large studies have examined the clinical features and outcomes of molecularly annotated patients with PMF. […] Patients with CALR mutation were younger, had higher platelet counts, and a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. […] The favorable effect of CALR mutations on survival and the poor survival of triple negative patients as compared to JAK2/MPL-mutated patients has also been shown in the post-allogeneic HCT setting in a German study. […] The resulting risk categories were very low (score 0), low (score 1), intermediate (score 2-3), high (score 4-5), and very high (score 6). […] A high molecular risk (HMR) signature was recently developed for patients with PMF in a multi-national study of 879 patients. […] Based on these findings, PMF patients with mutations in any of these five genes were identified as belonging to a HMR category. […] The ability of the GPSS to effectively discriminate between higher (high/intermediate-2) and lower (low/intermediate-1) risk categories in terms of both survival and LFS was validated in an independent cohort of 183 patients with PMF.

• #14 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Two large studies have examined the clinical features and outcomes of molecularly annotated patients with PMF. […] Patients with CALR mutation were younger, had higher platelet counts, and a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. […] The favorable effect of CALR mutations on survival and the poor survival of triple negative patients as compared to JAK2/MPL-mutated patients has also been shown in the post-allogeneic HCT setting in a German study. […] The resulting risk categories were very low (score 0), low (score 1), intermediate (score 2-3), high (score 4-5), and very high (score 6). […] A high molecular risk (HMR) signature was recently developed for patients with PMF in a multi-national study of 879 patients. […] Based on these findings, PMF patients with mutations in any of these five genes were identified as belonging to a HMR category. […] The ability of the GPSS to effectively discriminate between higher (high/intermediate-2) and lower (low/intermediate-1) risk categories in terms of both survival and LFS was validated in an independent cohort of 183 patients with PMF.

• #15 The number of prognostically detrimental mutations and prognosis in primary myelofibrosis: an international study of 797 patients | Leukemia

  https://www.nature.com/articles/leu201476

  We recently defined a high-molecular risk category (HMR) in primary myelofibrosis (PMF), based on the presence of at least one of the five prognostically detrimental mutated genes (ASXL1, EZH2, SRSF2 and IDH1/2). […] The presence of two or more mutations predicted the worst survival: median 2.6 years (hazard ratio (HR) 3.8, 95% confidence interval (CI) 2.6-5.7) vs 7.0 years (HR 1.9, 95% CI 1.4-2.6) for one mutation vs 12.3 years for no mutations. […] Two or more mutations were also associated with shortened leukemia-free survival (HR 6.2, 95% CI 3.5-10.7), also Mayo validated. […] We conclude that the number of prognostically detrimental mutations provides added value in the combined molecular and clinical prognostication of PMF.

• #16 The number of prognostically detrimental mutations and prognosis in primary myelofibrosis: an international study of 797 patients | Leukemia

  https://www.nature.com/articles/leu201476

  We recently defined a high-molecular risk category (HMR) in primary myelofibrosis (PMF), based on the presence of at least one of the five prognostically detrimental mutated genes (ASXL1, EZH2, SRSF2 and IDH1/2). […] The presence of two or more mutations predicted the worst survival: median 2.6 years (hazard ratio (HR) 3.8, 95% confidence interval (CI) 2.6-5.7) vs 7.0 years (HR 1.9, 95% CI 1.4-2.6) for one mutation vs 12.3 years for no mutations. […] Two or more mutations were also associated with shortened leukemia-free survival (HR 6.2, 95% CI 3.5-10.7), also Mayo validated. […] We conclude that the number of prognostically detrimental mutations provides added value in the combined molecular and clinical prognostication of PMF.

• #17 The number of prognostically detrimental mutations and prognosis in primary myelofibrosis: an international study of 797 patients | Leukemia

  https://www.nature.com/articles/leu201476

  We recently defined a high-molecular risk category (HMR) in primary myelofibrosis (PMF), based on the presence of at least one of the five prognostically detrimental mutated genes (ASXL1, EZH2, SRSF2 and IDH1/2). […] The presence of two or more mutations predicted the worst survival: median 2.6 years (hazard ratio (HR) 3.8, 95% confidence interval (CI) 2.6-5.7) vs 7.0 years (HR 1.9, 95% CI 1.4-2.6) for one mutation vs 12.3 years for no mutations. […] Two or more mutations were also associated with shortened leukemia-free survival (HR 6.2, 95% CI 3.5-10.7), also Mayo validated. […] We conclude that the number of prognostically detrimental mutations provides added value in the combined molecular and clinical prognostication of PMF.

• #18 Nationwide prospective survey of secondary myelofibrosis in Japan: superiority of DIPSS-plus to MYSEC-PM as a survival risk model | Blood Cancer Journal

  https://www.nature.com/articles/s41408-023-00869-9

  Myelofibrosis (MF) is divided into two categories, namely primary MF (PMF) and secondary MF arising from essential thrombocythemia (ET) or polycythemia vera (PV), so-called PET-MF or PPV-MF, respectively. […] However, discrepancies in the application of PMF prognostic scoring systems, such as the International Prognostic Scoring System (IPSS) and dynamic IPSS (DIPSS) for patients with PET/PPV-MF have been reported, and the prognostic scoring systems for PET/PPV-MF has been developed. […] We determined if the known prognostic factors used in the IPSS, DIPSS, DIPSS-plus, and the myelofibrosis secondary to PV and ET prognostic model (MYSEC-PM) were predictive of shortened survival in patients with PET/PPV-MF. […] This suggests that these three factors have a stronger impact on survival than other factors.

• #19 How Long Will I Live With Myelofibrosis? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/articles/understanding-mf-prognosis

  Around 10-20% of people with myelofibrosis will progress to acute myeloid leukemia. This type of leukemia is challenging to treat and has a significant impact on a persons life. For this reason, myelofibrosis prognosis will be directly linked to the patients risk of progressing to leukemia. […] A helpful tool for doctors to assess risk is the DIPSS dynamic international prognostic scoring system, which takes into account the following factors: Age (65 or older), Symptoms (non-specific indicators like fever, fatigue, appetite loss, etc), Anemia, High white blood cell count (leukocytosis), Circulating immature blood cells (blasts), Need for transfusions, Genetic mutations in the bone marrow cells. […] These risk factors help quickly assess a patient to calculate an approximate life expectancy. However, doctors consider other factors when meeting patients and learning more about their lifestyle, habits, family history, and overall health.

• #20 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  The main strength of the DIPSS is its applicability at any time during the PMF clinical course, emphasizing how anemia is often the earlier risk factor acquired during follow-up, manifesting myelodepletion and representative of disease progression. […] The same working group has proposed a further implementation of this score, called MIPSS70+, where the cytogenetic alterations are weighted. […] In both the training and validation cohorts, MIPSS70 and MIPSS70+ confirmed the potential to predict an inferior OS and a higher incidence of leukemic progression in high-risk patients. […] This score proves that genetic parameters could be enough to define the disease and its prognosis, but it also has the limitation of the difficulty of obtaining these data. […] The score was independently validated and is now routinely used in daily practice.

• #21 How Long Will I Live With Myelofibrosis? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/articles/understanding-mf-prognosis

  Around 10-20% of people with myelofibrosis will progress to acute myeloid leukemia. This type of leukemia is challenging to treat and has a significant impact on a persons life. For this reason, myelofibrosis prognosis will be directly linked to the patients risk of progressing to leukemia. […] A helpful tool for doctors to assess risk is the DIPSS dynamic international prognostic scoring system, which takes into account the following factors: Age (65 or older), Symptoms (non-specific indicators like fever, fatigue, appetite loss, etc), Anemia, High white blood cell count (leukocytosis), Circulating immature blood cells (blasts), Need for transfusions, Genetic mutations in the bone marrow cells. […] These risk factors help quickly assess a patient to calculate an approximate life expectancy. However, doctors consider other factors when meeting patients and learning more about their lifestyle, habits, family history, and overall health.

• #22 How Long Will I Live With Myelofibrosis? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/articles/understanding-mf-prognosis

  When a risk factor is present, the scoring system will add one point, and the total score will determine the risk group and is related to an approximate life expectancy: 0 points = low-risk (15.4 years), 1 point = intermediate-1-risk (6.5 years), 2-3 points = intermediate-2-risk (2.9 years), 4 or more points = high-risk (1.3 years). […] It is important to remember that this is not a definite prediction; it is based on statistics, and this type of information takes a long time to gather and is continuously updated. Many ongoing clinical trials and new treatments are being studied to extend life expectancy and improve the quality of life in patients with myelofibrosis. […] A prognostic score helps doctors select the best treatment for each patient. […] This is why it is very important to continue active research on high-risk myelofibrosis patients so that their progression can be prevented one day.

• #23 Myelofibrosis prognosis: Life expectancy, treatment options & more

  https://www.medicalnewstoday.com/articles/myelofibrosis-prognosis

  The outlook for someone with myelofibrosis depends on factors such as age, blood cell counts, and symptoms. For some people, the condition progresses more rapidly than others. […] Therefore, the MF outlook varies significantly. […] Predicting the life expectancy of someone with MF is challenging. […] The approximate life expectancy for those with MF is as follows: 15.4 years for low risk individuals, 6.5 years for intermediate-1 (INT-1) risk individuals, 2.9 years for INT-2 risk individuals, 1.3 years for high risk individuals. […] However, while outcome data can illustrate how people with MF have fared in response to treatments, it may not precisely predict how an individual will respond. […] A person’s myelofibrosis prognosis depends on various factors, and some people remain symptom-free for many years. […] However, for those with risk factors such as being over 65 years old, having whole-body symptoms or anemia, and having specific genetic abnormalities, the outlook may be less favorable.

• #24 Nationwide prospective survey of secondary myelofibrosis in Japan: superiority of DIPSS-plus to MYSEC-PM as a survival risk model | Blood Cancer Journal

  https://www.nature.com/articles/s41408-023-00869-9

  The overall predictive performance of the models was evaluated using Harrells C-statistics. The DIPSS-plus had the highest C-index (0.887), followed by IPSS and DIPSS. Meanwhile, the MYSEC-PM had the lowest C-index (0.806). […] The superiority of the DIPSS-plus over other models may be due to the greater impact of karyotype on prognosis. […] In conclusion, the prognostic models for PMF, such as the DIPSS-plus, which contain karyotype with a strong impact on survival, may be useful for PET/PPV-MF patients in identifying HCT candidates and predicting the prognosis of patients even in the MYSEC-PM era.

• #25 Nationwide prospective survey of secondary myelofibrosis in Japan: superiority of DIPSS-plus to MYSEC-PM as a survival risk model | Blood Cancer Journal

  https://www.nature.com/articles/s41408-023-00869-9

  The overall predictive performance of the models was evaluated using Harrells C-statistics. The DIPSS-plus had the highest C-index (0.887), followed by IPSS and DIPSS. Meanwhile, the MYSEC-PM had the lowest C-index (0.806). […] The superiority of the DIPSS-plus over other models may be due to the greater impact of karyotype on prognosis. […] In conclusion, the prognostic models for PMF, such as the DIPSS-plus, which contain karyotype with a strong impact on survival, may be useful for PET/PPV-MF patients in identifying HCT candidates and predicting the prognosis of patients even in the MYSEC-PM era.

• #26 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  The main strength of the DIPSS is its applicability at any time during the PMF clinical course, emphasizing how anemia is often the earlier risk factor acquired during follow-up, manifesting myelodepletion and representative of disease progression. […] The same working group has proposed a further implementation of this score, called MIPSS70+, where the cytogenetic alterations are weighted. […] In both the training and validation cohorts, MIPSS70 and MIPSS70+ confirmed the potential to predict an inferior OS and a higher incidence of leukemic progression in high-risk patients. […] This score proves that genetic parameters could be enough to define the disease and its prognosis, but it also has the limitation of the difficulty of obtaining these data. […] The score was independently validated and is now routinely used in daily practice.

• #27 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  The main strength of the DIPSS is its applicability at any time during the PMF clinical course, emphasizing how anemia is often the earlier risk factor acquired during follow-up, manifesting myelodepletion and representative of disease progression. […] The same working group has proposed a further implementation of this score, called MIPSS70+, where the cytogenetic alterations are weighted. […] In both the training and validation cohorts, MIPSS70 and MIPSS70+ confirmed the potential to predict an inferior OS and a higher incidence of leukemic progression in high-risk patients. […] This score proves that genetic parameters could be enough to define the disease and its prognosis, but it also has the limitation of the difficulty of obtaining these data. […] The score was independently validated and is now routinely used in daily practice.

• #28 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  The main strength of the DIPSS is its applicability at any time during the PMF clinical course, emphasizing how anemia is often the earlier risk factor acquired during follow-up, manifesting myelodepletion and representative of disease progression. […] The same working group has proposed a further implementation of this score, called MIPSS70+, where the cytogenetic alterations are weighted. […] In both the training and validation cohorts, MIPSS70 and MIPSS70+ confirmed the potential to predict an inferior OS and a higher incidence of leukemic progression in high-risk patients. […] This score proves that genetic parameters could be enough to define the disease and its prognosis, but it also has the limitation of the difficulty of obtaining these data. […] The score was independently validated and is now routinely used in daily practice.

• #29

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  Myelofibrosis (MF) includes prefibrotic primary MF (pre-PMF), overt-PMF and secondary MF (SMF). Median overall survival (OS) of pre-PMF, overt-PMF and SMF patients is around 14 years, seven and nine years, respectively. […] Discoveries on the impact of the biological architecture on OS have led to the design of integrated scores to predict survival in PMF. […] Prognostic models guide prediction of OS and treatment planning in MF, therefore, their timely application is critical in the personalized approach of MF patients. […] Patients affected by MF have a significantly reduced outcome, with a median OS of 14 years, seven and nine years in pre-PMF, overt-PMF and SMF, respectively. […] Nonetheless, in recent years improvement in prognosis has been registered. […] The MYSEC cohort, that represents to date the largest dataset of SMF patients, allowed to generate a specific clinical-molecular prognostic score, the MYSEC-Prognostic Model (MYSEC-PM).

• #30

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  The MYSEC-PM could be easily calculated by a nomogram depicted on the original paper and by an online application. […] The impact of BMF (grade 2 vs 3) has been investigated in a more recent subanalysis of the MYSEC cohort: out of 805 SMF, 34% had a grade 3 BMF at evolution. […] The updated EBMT/ELN guidelines suggest considering low and some intermediate risk MTSS patients for allo-SCT. […] The majority of MF patients are not suitable for allo-SCT and mostly receive JAKis. […] In the recent years, outcome of MF patients has improved, due to early diagnosis, use of JAKis and improved management of candidates to allo-SCT. […] The increased knowledge on the biological landscape of PMF has broadened the number of available survival models, that should be applied simultaneously for a more personalized definition of outcome, especially in younger patients.

• #31 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Two large studies have examined the clinical features and outcomes of molecularly annotated patients with PMF. […] Patients with CALR mutation were younger, had higher platelet counts, and a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. […] The favorable effect of CALR mutations on survival and the poor survival of triple negative patients as compared to JAK2/MPL-mutated patients has also been shown in the post-allogeneic HCT setting in a German study. […] The resulting risk categories were very low (score 0), low (score 1), intermediate (score 2-3), high (score 4-5), and very high (score 6). […] A high molecular risk (HMR) signature was recently developed for patients with PMF in a multi-national study of 879 patients. […] Based on these findings, PMF patients with mutations in any of these five genes were identified as belonging to a HMR category. […] The ability of the GPSS to effectively discriminate between higher (high/intermediate-2) and lower (low/intermediate-1) risk categories in terms of both survival and LFS was validated in an independent cohort of 183 patients with PMF.

• #32 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Therefore, the RR6 score applied after 6 months of therapy represents a turning point in establishing an early shift to other treatments, sometimes radical, such as to HSCT. […] In this context, AIPSS-MF represented the first milestone that, when validated on large external courts, will allow the exploration of new frontiers. […] Currently, several prognostic models can identify the patients with the worst outcome. […] The clinicians challenge is to approach each patient in each phase of the disease carefully, extrapolating the results of the prognostic scores and thus obtaining critical information to guarantee the best therapeutic approach to best manage MF.

• #33 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Therefore, the RR6 score applied after 6 months of therapy represents a turning point in establishing an early shift to other treatments, sometimes radical, such as to HSCT. […] In this context, AIPSS-MF represented the first milestone that, when validated on large external courts, will allow the exploration of new frontiers. […] Currently, several prognostic models can identify the patients with the worst outcome. […] The clinicians challenge is to approach each patient in each phase of the disease carefully, extrapolating the results of the prognostic scores and thus obtaining critical information to guarantee the best therapeutic approach to best manage MF.

• #34 A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis

  https://www.mdpi.com/2072-6694/15/20/5027

  The STR-PM may identify patients at higher risk of failure with ruxolitinib monotherapy who should be considered for alternative frontline strategies. […] The aims of this study were to investigate predictors of early ruxolitinib discontinuation and death on therapy in 889 MF patients and to create a prognostic score which could help to identify those patients at higher risk of failure of ruxolitinib monotherapy. […] The probability of remaining on ruxolitinib may be predicted by the STR-PM, which consists of several factors, including ruxolitinib starting dose ≥10 mg BID, baseline hemoglobin ≥10 g/dL, baseline platelet count ≥100 × 10^9/L, diagnosis of post-Polycythemia Vera/Essential Thrombocythemia MF, and achievement of a spleen response at 3 months. […] The most important clinical value of this score is in the early recognition of patients who are at risk of suboptimal response to therapy and who would be candidates to prepare for enrollment in clinical studies, etc., upon start of ruxolitinib treatment.

• #35 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Two large studies have examined the clinical features and outcomes of molecularly annotated patients with PMF. […] Patients with CALR mutation were younger, had higher platelet counts, and a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. […] The favorable effect of CALR mutations on survival and the poor survival of triple negative patients as compared to JAK2/MPL-mutated patients has also been shown in the post-allogeneic HCT setting in a German study. […] The resulting risk categories were very low (score 0), low (score 1), intermediate (score 2-3), high (score 4-5), and very high (score 6). […] A high molecular risk (HMR) signature was recently developed for patients with PMF in a multi-national study of 879 patients. […] Based on these findings, PMF patients with mutations in any of these five genes were identified as belonging to a HMR category. […] The ability of the GPSS to effectively discriminate between higher (high/intermediate-2) and lower (low/intermediate-1) risk categories in terms of both survival and LFS was validated in an independent cohort of 183 patients with PMF.

• #36 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Two large studies have examined the clinical features and outcomes of molecularly annotated patients with PMF. […] Patients with CALR mutation were younger, had higher platelet counts, and a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. […] The favorable effect of CALR mutations on survival and the poor survival of triple negative patients as compared to JAK2/MPL-mutated patients has also been shown in the post-allogeneic HCT setting in a German study. […] The resulting risk categories were very low (score 0), low (score 1), intermediate (score 2-3), high (score 4-5), and very high (score 6). […] A high molecular risk (HMR) signature was recently developed for patients with PMF in a multi-national study of 879 patients. […] Based on these findings, PMF patients with mutations in any of these five genes were identified as belonging to a HMR category. […] The ability of the GPSS to effectively discriminate between higher (high/intermediate-2) and lower (low/intermediate-1) risk categories in terms of both survival and LFS was validated in an independent cohort of 183 patients with PMF.

• #37 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Currently, prognostication in primary myelofibrosis (PMF) relies on the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS) and DIPSS-plus, which incorporate age, blood counts, constitutional symptoms, circulating blasts, red cell transfusion need and karyotype. […] The number of prognostically detrimental mutations may affect both survival and response to ruxolitinib, which has significant implications for clinical decision making. […] Recently, the Janus kinase (JAK) 1/2 inhibitor, ruxolitinib, approved by the Food and Drug Administration (FDA) for the treatment of patients with intermediate or high risk patients with PMF, post-PV or post-ET MF, has demonstrated a survival benefit for these poor risk categories of patients in randomized, controlled clinical trials. […] A low, rather than high, JAK2 V617F allele burden has been associated with inferior survival and LFS in PMF.

• #38 Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements

  https://www.mdpi.com/2072-6694/16/3/514

  Myelofibrosis (MF) is an essential element of primary myelofibrosis, whereas secondary MF may develop in the advanced stages of other myeloid neoplasms, especially polycythemia vera and essential thrombocythemia. Over the last two decades, advances in molecular diagnostic techniques, particularly the integration of next-generation sequencing in clinical laboratories, have revolutionized the diagnosis, classification, and clinical decision making of myelofibrosis. […] Prognostic prediction plays a pivotal role in guiding the treatment of myelofibrosis. Mutation profiles and cytogenetic abnormalities have been integrated into advanced prognostic scoring systems and personalized risk stratification for MF. […] The evolving landscape of prognostic systems for myelofibrosis underscores the increasing recognition of complex clinical, cytogenetic, and molecular factors for predicting disease outcomes. The prognostic value of HMR mutations in MIPSS70+ V2.0 has been confirmed by recent clinical study results.

• #39 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Currently, prognostication in primary myelofibrosis (PMF) relies on the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS) and DIPSS-plus, which incorporate age, blood counts, constitutional symptoms, circulating blasts, red cell transfusion need and karyotype. […] The number of prognostically detrimental mutations may affect both survival and response to ruxolitinib, which has significant implications for clinical decision making. […] Recently, the Janus kinase (JAK) 1/2 inhibitor, ruxolitinib, approved by the Food and Drug Administration (FDA) for the treatment of patients with intermediate or high risk patients with PMF, post-PV or post-ET MF, has demonstrated a survival benefit for these poor risk categories of patients in randomized, controlled clinical trials. […] A low, rather than high, JAK2 V617F allele burden has been associated with inferior survival and LFS in PMF.

• #40 Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements

  https://www.mdpi.com/2072-6694/16/3/514

  It is undeniable that the current molecular genetic profiling results fall short of capturing all prognostically significant factors in MF. […] The treatment strategy depends on the clinical presentation, risk stratification, prediction of prognosis, and transplant-specific risk (MTSS) to select patients eligible for HSCT. […] The approval of JAK inhibitors and other novel agents has significantly changed the treatment landscape of MF. […] Several studies have demonstrated that additional non-driver mutations may predict clinical response to JAK inhibitor treatment. […] Clonal evolution during the treatment process indicates the need to monitor the mutation profile in the follow-up of patients treated with JAK inhibitors. […] The prospect of specific therapies that induce complete molecular genetic remission, analogous to tyrosine kinase inhibitor therapy for BCR::ABL1-positive CML, remains a compelling approach in the pursuit of the best clinical outcomes for MF.

• #41 Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements

  https://www.mdpi.com/2072-6694/16/3/514

  It is undeniable that the current molecular genetic profiling results fall short of capturing all prognostically significant factors in MF. […] The treatment strategy depends on the clinical presentation, risk stratification, prediction of prognosis, and transplant-specific risk (MTSS) to select patients eligible for HSCT. […] The approval of JAK inhibitors and other novel agents has significantly changed the treatment landscape of MF. […] Several studies have demonstrated that additional non-driver mutations may predict clinical response to JAK inhibitor treatment. […] Clonal evolution during the treatment process indicates the need to monitor the mutation profile in the follow-up of patients treated with JAK inhibitors. […] The prospect of specific therapies that induce complete molecular genetic remission, analogous to tyrosine kinase inhibitor therapy for BCR::ABL1-positive CML, remains a compelling approach in the pursuit of the best clinical outcomes for MF.

• #42 Prognosis for myelofibrosis (MF) | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/myelofibrosis/myelofibrosis-prognosis/

  Myelofibrosis is different for different people. For some its slow growing and may not need treatment right away. Others may need treatment more quickly. […] Your prognosis (what may happen in the future) is personal to you. It will depend on lots of things: your blood counts, your symptoms, your age, your overall health, including other conditions you may have, the genetic mutation thats causing the MF. […] Based on these things, your doctor may calculate a risk score. There are a few different systems in use but generally they come up with a risk score of low, intermediate-1, intermediate-2 or high. This gives doctors an idea of your prognosis and helps them decide what treatment you need. If your risk score is low or intermediate-1, you may not need treatment at all. […] Prefibrotic myelofibrosis (MF) usually has a lower risk score than overt MF, but your doctor will take everything into account and recommend care based on your individual needs. […] Its important to understand that statistics about survival are very general they dont reflect your personal prognosis. Also, your prognosis can change over time because it is affected by things like starting treatment or changing to a different treatment.

• #43

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  Myelofibrosis (MF) includes prefibrotic primary MF (pre-PMF), overt-PMF and secondary MF (SMF). Median overall survival (OS) of pre-PMF, overt-PMF and SMF patients is around 14 years, seven and nine years, respectively. […] Discoveries on the impact of the biological architecture on OS have led to the design of integrated scores to predict survival in PMF. […] Prognostic models guide prediction of OS and treatment planning in MF, therefore, their timely application is critical in the personalized approach of MF patients. […] Patients affected by MF have a significantly reduced outcome, with a median OS of 14 years, seven and nine years in pre-PMF, overt-PMF and SMF, respectively. […] Nonetheless, in recent years improvement in prognosis has been registered. […] The MYSEC cohort, that represents to date the largest dataset of SMF patients, allowed to generate a specific clinical-molecular prognostic score, the MYSEC-Prognostic Model (MYSEC-PM).

• #44

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  Myelofibrosis (MF) includes prefibrotic primary MF (pre-PMF), overt-PMF and secondary MF (SMF). Median overall survival (OS) of pre-PMF, overt-PMF and SMF patients is around 14 years, seven and nine years, respectively. […] Discoveries on the impact of the biological architecture on OS have led to the design of integrated scores to predict survival in PMF. […] Prognostic models guide prediction of OS and treatment planning in MF, therefore, their timely application is critical in the personalized approach of MF patients. […] Patients affected by MF have a significantly reduced outcome, with a median OS of 14 years, seven and nine years in pre-PMF, overt-PMF and SMF, respectively. […] Nonetheless, in recent years improvement in prognosis has been registered. […] The MYSEC cohort, that represents to date the largest dataset of SMF patients, allowed to generate a specific clinical-molecular prognostic score, the MYSEC-Prognostic Model (MYSEC-PM).

• #45 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  The mortality risk of secondary myelofibrosis (sMF) is higher than in patients diagnosed with primary myelofibrosis (PMF). […] Other risk factors can contribute to a poorer prognosis of primary myelofibrosis. […] Transformation into leukemia occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. […] Approximately 70% of patients with primary myelofibrosis show an increased density of the bone marrow’s smallest blood vessels, which is also associated with poor survival. […] To help predict your individual outlook, doctors use risk stratification scores. […] The only potentially curative treatment for myelofibrosis is a stem cell transplant. The cure rate with this treatment can be between 30% and 65%, but this comes at an increased mortality risk. The 3- to 5-year survival rate for patients with myelofibrosis who underwent a stem cell transplant ranges from 45% to 70%; relapse and transplant-related mortality typically occur within the first 2 years after treatment.

• #46

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  Myelofibrosis (MF) includes prefibrotic primary MF (pre-PMF), overt-PMF and secondary MF (SMF). Median overall survival (OS) of pre-PMF, overt-PMF and SMF patients is around 14 years, seven and nine years, respectively. […] Discoveries on the impact of the biological architecture on OS have led to the design of integrated scores to predict survival in PMF. […] Prognostic models guide prediction of OS and treatment planning in MF, therefore, their timely application is critical in the personalized approach of MF patients. […] Patients affected by MF have a significantly reduced outcome, with a median OS of 14 years, seven and nine years in pre-PMF, overt-PMF and SMF, respectively. […] Nonetheless, in recent years improvement in prognosis has been registered. […] The MYSEC cohort, that represents to date the largest dataset of SMF patients, allowed to generate a specific clinical-molecular prognostic score, the MYSEC-Prognostic Model (MYSEC-PM).

• #47 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  The mortality risk of secondary myelofibrosis (sMF) is higher than in patients diagnosed with primary myelofibrosis (PMF). […] Other risk factors can contribute to a poorer prognosis of primary myelofibrosis. […] Transformation into leukemia occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. […] Approximately 70% of patients with primary myelofibrosis show an increased density of the bone marrow’s smallest blood vessels, which is also associated with poor survival. […] To help predict your individual outlook, doctors use risk stratification scores. […] The only potentially curative treatment for myelofibrosis is a stem cell transplant. The cure rate with this treatment can be between 30% and 65%, but this comes at an increased mortality risk. The 3- to 5-year survival rate for patients with myelofibrosis who underwent a stem cell transplant ranges from 45% to 70%; relapse and transplant-related mortality typically occur within the first 2 years after treatment.

• #48 How Long Will I Live With Myelofibrosis? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/articles/understanding-mf-prognosis

  Around 10-20% of people with myelofibrosis will progress to acute myeloid leukemia. This type of leukemia is challenging to treat and has a significant impact on a persons life. For this reason, myelofibrosis prognosis will be directly linked to the patients risk of progressing to leukemia. […] A helpful tool for doctors to assess risk is the DIPSS dynamic international prognostic scoring system, which takes into account the following factors: Age (65 or older), Symptoms (non-specific indicators like fever, fatigue, appetite loss, etc), Anemia, High white blood cell count (leukocytosis), Circulating immature blood cells (blasts), Need for transfusions, Genetic mutations in the bone marrow cells. […] These risk factors help quickly assess a patient to calculate an approximate life expectancy. However, doctors consider other factors when meeting patients and learning more about their lifestyle, habits, family history, and overall health.

• #49 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  The mortality risk of secondary myelofibrosis (sMF) is higher than in patients diagnosed with primary myelofibrosis (PMF). […] Other risk factors can contribute to a poorer prognosis of primary myelofibrosis. […] Transformation into leukemia occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. […] Approximately 70% of patients with primary myelofibrosis show an increased density of the bone marrow’s smallest blood vessels, which is also associated with poor survival. […] To help predict your individual outlook, doctors use risk stratification scores. […] The only potentially curative treatment for myelofibrosis is a stem cell transplant. The cure rate with this treatment can be between 30% and 65%, but this comes at an increased mortality risk. The 3- to 5-year survival rate for patients with myelofibrosis who underwent a stem cell transplant ranges from 45% to 70%; relapse and transplant-related mortality typically occur within the first 2 years after treatment.

• #50 Prognosis of primary myelofibrosis in the genomic era

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4987499/

  Currently, prognostication in primary myelofibrosis (PMF) relies on the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS) and DIPSS-plus, which incorporate age, blood counts, constitutional symptoms, circulating blasts, red cell transfusion need and karyotype. […] The number of prognostically detrimental mutations may affect both survival and response to ruxolitinib, which has significant implications for clinical decision making. […] Recently, the Janus kinase (JAK) 1/2 inhibitor, ruxolitinib, approved by the Food and Drug Administration (FDA) for the treatment of patients with intermediate or high risk patients with PMF, post-PV or post-ET MF, has demonstrated a survival benefit for these poor risk categories of patients in randomized, controlled clinical trials. […] A low, rather than high, JAK2 V617F allele burden has been associated with inferior survival and LFS in PMF.

• #51 Myelofibrosis: Prognosis and Life Expectancy

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

  People who dont meet the above criteria, excluding age, are considered in the low risk category and have a median survival of over 15 years. […] A 2022 study that examined the effect of treatment on MF also found that treating with Janus Kinase (JAK) inhibitors led to a median overall survival of 84 months, which is an improvement from about 64 months a decade ago. […] A persons outlook with myelofibrosis can vary from around 1 year to more than 15 years, depending on individual risk factors and disease progression. […] Treatment may help reduce symptoms, slow disease progression, and improve your quality of life.

• #52 A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis

  https://www.mdpi.com/2072-6694/15/20/5027

  A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis […] Despite significant clinical activity, 50% to 70% of patients discontinue ruxolitinib within 3 to 5 years. […] The identification of patients who are more likely to discontinue it early has now become of paramount practical importance, given the availability of new drugs that are either approved (i.e., fedratinib, pacritinib, and momelotinib) or undergoing advanced clinical investigation for patients with a suboptimal response or ruxolitinib resistance (i.e., navitoclax, pelabresib, and imetelstat). […] Results confirm that prolonged ruxolitinib administration is associated with improved OS when compared to earlier discontinuation. […] Overall survival (OS) was significantly longer in LTR pts (p = 0.002).

• #53 A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis

  https://www.mdpi.com/2072-6694/15/20/5027

  A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis […] Despite significant clinical activity, 50% to 70% of patients discontinue ruxolitinib within 3 to 5 years. […] The identification of patients who are more likely to discontinue it early has now become of paramount practical importance, given the availability of new drugs that are either approved (i.e., fedratinib, pacritinib, and momelotinib) or undergoing advanced clinical investigation for patients with a suboptimal response or ruxolitinib resistance (i.e., navitoclax, pelabresib, and imetelstat). […] Results confirm that prolonged ruxolitinib administration is associated with improved OS when compared to earlier discontinuation. […] Overall survival (OS) was significantly longer in LTR pts (p = 0.002).

• #54 Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements

  https://www.mdpi.com/2072-6694/16/3/514

  It is undeniable that the current molecular genetic profiling results fall short of capturing all prognostically significant factors in MF. […] The treatment strategy depends on the clinical presentation, risk stratification, prediction of prognosis, and transplant-specific risk (MTSS) to select patients eligible for HSCT. […] The approval of JAK inhibitors and other novel agents has significantly changed the treatment landscape of MF. […] Several studies have demonstrated that additional non-driver mutations may predict clinical response to JAK inhibitor treatment. […] Clonal evolution during the treatment process indicates the need to monitor the mutation profile in the follow-up of patients treated with JAK inhibitors. […] The prospect of specific therapies that induce complete molecular genetic remission, analogous to tyrosine kinase inhibitor therapy for BCR::ABL1-positive CML, remains a compelling approach in the pursuit of the best clinical outcomes for MF.

• #55 A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis

  https://www.mdpi.com/2072-6694/15/20/5027

  A Prognostic Model to Predict Ruxolitinib Discontinuation and Death in Patients with Myelofibrosis […] Despite significant clinical activity, 50% to 70% of patients discontinue ruxolitinib within 3 to 5 years. […] The identification of patients who are more likely to discontinue it early has now become of paramount practical importance, given the availability of new drugs that are either approved (i.e., fedratinib, pacritinib, and momelotinib) or undergoing advanced clinical investigation for patients with a suboptimal response or ruxolitinib resistance (i.e., navitoclax, pelabresib, and imetelstat). […] Results confirm that prolonged ruxolitinib administration is associated with improved OS when compared to earlier discontinuation. […] Overall survival (OS) was significantly longer in LTR pts (p = 0.002).

• #56 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  The mortality risk of secondary myelofibrosis (sMF) is higher than in patients diagnosed with primary myelofibrosis (PMF). […] Other risk factors can contribute to a poorer prognosis of primary myelofibrosis. […] Transformation into leukemia occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. […] Approximately 70% of patients with primary myelofibrosis show an increased density of the bone marrow’s smallest blood vessels, which is also associated with poor survival. […] To help predict your individual outlook, doctors use risk stratification scores. […] The only potentially curative treatment for myelofibrosis is a stem cell transplant. The cure rate with this treatment can be between 30% and 65%, but this comes at an increased mortality risk. The 3- to 5-year survival rate for patients with myelofibrosis who underwent a stem cell transplant ranges from 45% to 70%; relapse and transplant-related mortality typically occur within the first 2 years after treatment.

• #57 What Is Myelofibrosis Life Expectancy? – HealthTree for Myelofibrosis

  https://healthtree.org/myelofibrosis/community/what-is-the-survival-rate-for-myelofibrosis

  The mortality risk of secondary myelofibrosis (sMF) is higher than in patients diagnosed with primary myelofibrosis (PMF). […] Other risk factors can contribute to a poorer prognosis of primary myelofibrosis. […] Transformation into leukemia occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. […] Approximately 70% of patients with primary myelofibrosis show an increased density of the bone marrow’s smallest blood vessels, which is also associated with poor survival. […] To help predict your individual outlook, doctors use risk stratification scores. […] The only potentially curative treatment for myelofibrosis is a stem cell transplant. The cure rate with this treatment can be between 30% and 65%, but this comes at an increased mortality risk. The 3- to 5-year survival rate for patients with myelofibrosis who underwent a stem cell transplant ranges from 45% to 70%; relapse and transplant-related mortality typically occur within the first 2 years after treatment.

• #58

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  The MYSEC-PM could be easily calculated by a nomogram depicted on the original paper and by an online application. […] The impact of BMF (grade 2 vs 3) has been investigated in a more recent subanalysis of the MYSEC cohort: out of 805 SMF, 34% had a grade 3 BMF at evolution. […] The updated EBMT/ELN guidelines suggest considering low and some intermediate risk MTSS patients for allo-SCT. […] The majority of MF patients are not suitable for allo-SCT and mostly receive JAKis. […] In the recent years, outcome of MF patients has improved, due to early diagnosis, use of JAKis and improved management of candidates to allo-SCT. […] The increased knowledge on the biological landscape of PMF has broadened the number of available survival models, that should be applied simultaneously for a more personalized definition of outcome, especially in younger patients.

• #59

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  The MYSEC-PM could be easily calculated by a nomogram depicted on the original paper and by an online application. […] The impact of BMF (grade 2 vs 3) has been investigated in a more recent subanalysis of the MYSEC cohort: out of 805 SMF, 34% had a grade 3 BMF at evolution. […] The updated EBMT/ELN guidelines suggest considering low and some intermediate risk MTSS patients for allo-SCT. […] The majority of MF patients are not suitable for allo-SCT and mostly receive JAKis. […] In the recent years, outcome of MF patients has improved, due to early diagnosis, use of JAKis and improved management of candidates to allo-SCT. […] The increased knowledge on the biological landscape of PMF has broadened the number of available survival models, that should be applied simultaneously for a more personalized definition of outcome, especially in younger patients.

• #60 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Therefore, the RR6 score applied after 6 months of therapy represents a turning point in establishing an early shift to other treatments, sometimes radical, such as to HSCT. […] In this context, AIPSS-MF represented the first milestone that, when validated on large external courts, will allow the exploration of new frontiers. […] Currently, several prognostic models can identify the patients with the worst outcome. […] The clinicians challenge is to approach each patient in each phase of the disease carefully, extrapolating the results of the prognostic scores and thus obtaining critical information to guarantee the best therapeutic approach to best manage MF.

• #61 Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10053868/

  Therefore, the RR6 score applied after 6 months of therapy represents a turning point in establishing an early shift to other treatments, sometimes radical, such as to HSCT. […] In this context, AIPSS-MF represented the first milestone that, when validated on large external courts, will allow the exploration of new frontiers. […] Currently, several prognostic models can identify the patients with the worst outcome. […] The clinicians challenge is to approach each patient in each phase of the disease carefully, extrapolating the results of the prognostic scores and thus obtaining critical information to guarantee the best therapeutic approach to best manage MF.

• #62

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  The MYSEC-PM could be easily calculated by a nomogram depicted on the original paper and by an online application. […] The impact of BMF (grade 2 vs 3) has been investigated in a more recent subanalysis of the MYSEC cohort: out of 805 SMF, 34% had a grade 3 BMF at evolution. […] The updated EBMT/ELN guidelines suggest considering low and some intermediate risk MTSS patients for allo-SCT. […] The majority of MF patients are not suitable for allo-SCT and mostly receive JAKis. […] In the recent years, outcome of MF patients has improved, due to early diagnosis, use of JAKis and improved management of candidates to allo-SCT. […] The increased knowledge on the biological landscape of PMF has broadened the number of available survival models, that should be applied simultaneously for a more personalized definition of outcome, especially in younger patients.

• #63

  https://link.springer.com/article/10.1007/s11899-024-00739-6

  The MYSEC-PM could be easily calculated by a nomogram depicted on the original paper and by an online application. […] The impact of BMF (grade 2 vs 3) has been investigated in a more recent subanalysis of the MYSEC cohort: out of 805 SMF, 34% had a grade 3 BMF at evolution. […] The updated EBMT/ELN guidelines suggest considering low and some intermediate risk MTSS patients for allo-SCT. […] The majority of MF patients are not suitable for allo-SCT and mostly receive JAKis. […] In the recent years, outcome of MF patients has improved, due to early diagnosis, use of JAKis and improved management of candidates to allo-SCT. […] The increased knowledge on the biological landscape of PMF has broadened the number of available survival models, that should be applied simultaneously for a more personalized definition of outcome, especially in younger patients.

• #64 Prognosis for myelofibrosis (MF) | Blood Cancer UK

  https://bloodcancer.org.uk/understanding-blood-cancer/myelofibrosis/myelofibrosis-prognosis/

  Myelofibrosis is different for different people. For some its slow growing and may not need treatment right away. Others may need treatment more quickly. […] Your prognosis (what may happen in the future) is personal to you. It will depend on lots of things: your blood counts, your symptoms, your age, your overall health, including other conditions you may have, the genetic mutation thats causing the MF. […] Based on these things, your doctor may calculate a risk score. There are a few different systems in use but generally they come up with a risk score of low, intermediate-1, intermediate-2 or high. This gives doctors an idea of your prognosis and helps them decide what treatment you need. If your risk score is low or intermediate-1, you may not need treatment at all. […] Prefibrotic myelofibrosis (MF) usually has a lower risk score than overt MF, but your doctor will take everything into account and recommend care based on your individual needs. […] Its important to understand that statistics about survival are very general they dont reflect your personal prognosis. Also, your prognosis can change over time because it is affected by things like starting treatment or changing to a different treatment.

Zobacz więcej