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What is the life expectancy for someone with myelofibrosis?

Myelofibrosis is aggressive cancer with a median survival rate of six years. A median is a midpoint, which means that some people live less than six years, and about the same number of people live longer than six years.

The prognosis for myelofibrosis varies widely depending on several factors:

  1. Disease Stage: Myelofibrosis has different stages, ranging from early-stage (low-risk) to advanced-stage (high-risk). Patients with early-stage disease generally have a better prognosis than those with advanced-stage disease.

  2. Age: Age can impact prognosis, with older individuals potentially facing more complications and challenges in managing the disease.

  3. Symptoms and Complications: The severity of symptoms and complications, such as an enlarged spleen or recurrent infections, can influence prognosis.

  4. Genetic Mutations: Specific genetic mutations, such as the presence of mutations in genes like JAK2, CALR, or MPL, can affect disease progression and response to treatment.

  5. Other Health Conditions: Other medical conditions (comorbidities) can complicate the management of myelofibrosis and impact overall health.

  6. Response to Treatment: Advances in treatment options, including targeted therapies and bone marrow transplantation, have improved outcomes for many individuals with myelofibrosis. How well a person responds to treatment can significantly affect their prognosis.

  7. Transformation to Acute Leukemia: In some cases, myelofibrosis can transform into acute leukemia, which typically has a poor prognosis.

During the past few decades, advancements in treatments for myelofibrosis have increased patient survival. Several sources report that the median survival for patients with primary myelofibrosis is between 3.5 and 5.5 years. The most common causes of death in patients with myelofibrosis include infection, hemorrhage, cardiac failure, mortality following splenectomy, and myelofibrosis advancing to acute myeloid leukemia (AML). Other reported causes of mortality include renal failure, hepatic failure, and thrombosis. Many causes of death are disease or treatment-related; however, others are comorbidities.

Life Expectancy Relative to Age

Although myelofibrosis can occur at any age, it primarily affects individuals older than 50 years, with a median age at diagnosis of approximately 65 years. Older age correlates with shorter survival in patients with myelofibrosis.

Researchers in a study published in 2018 analyzed the clinical and genetic markers predicting short-term (≤5 years) or long-term (20+ years) survival in patients with primary myelofibrosis. They reported that age older than 70 years independently predicted 5-year mortality, whereas patients age 70 or younger were more likely to survive beyond 20 years.

Life Expectancy Relative to Sex

Women with myelofibrosis are more likely than men to survive longer than 20 years after a diagnosis of myelofibrosis. Fewer than 20% of patients with myelofibrosis are predicted to survive longer than 10 years.

Life Expectancy Relative to Comorbidities

Because myelofibrosis affects primarily an older population, chronic comorbidities are often present, including hypertension, cardiovascular disease, dyslipidemia, other malignancies, diabetes, renal dysfunction, pulmonary disease, and hepatic disease.

Researchers in a 2022 study involving 668 patients with myelofibrosis identified the comorbidities linked with the most significant adverse effect on overall survival. These included hypertension, high cholesterol, smoking, and hepatitis C. Diabetes, renal dysfunction (kidney), and pulmonary disease also increased the risk of death.

Life Expectancy Relative to Myelofibrosis Type and Disease Progression

The risk for mortality in patients with polycythemia vera and essential thrombocythemia that progresses to secondary myelofibrosis is higher than in patients with primary myelofibrosis. 

Polycythemia vera and essential thrombocythemia are generally considered to be less aggressive myeloproliferative neoplasms than primary myelofibrosis; however, genetic mutational differences have been noted between patients with myelofibrosis secondary to Polycythemia vera or essential thrombocythemia and those with primary myelofibrosis. These mutational differences may affect disease progression to more aggressive forms, such as secondary myelofibrosis or even AML.

Leukemic transformation occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. Progression to blast phase disease correlates with patient mortality. Increased density of the bone marrow microvasculature, which occurs in approximately 70% of patients with primary myelofibrosis, is an independent risk factor for poor survival.

Life Expectancy Relative to Risk Stratification and Genetics

Several scoring systems are used for the risk stratification of patients with myelofibrosis: the DIPSS-plus; the Mutation-Enhanced International Prognostic Scoring System (MIPSS-70), used to evaluate potential candidates for allogeneic stem cell transplant; and the Genetically Inspired Prognostic Scoring System (GIPSS).

Life Expectancy According to DIPSS-plus

DIPSS-plus classifies patients with myelofibrosis into 4 risk categories: 

  • Low risk (0 points), with a median survival of 15.4 years
  • Intermediate-1 risk (1 point), with a median survival of 6.5 years
  • Intermediate-2 risk (2-3 points), with a median survival of 35 months (2.9 years)
  • High risk (4-6 points), with a median survival of 16 months (1.3 years)

Life Expectancy According to MIPSS70 

MIPSS70 classifies patients with myelofibrosis as being at low, intermediate, or high risk according to the following 3 genetic and 6 clinical risk factors5:

  • Absence of CALR type 1/like mutations
  • Presence of any high-molecular-risk mutation, such as in ASXL1, SRSF2, EZH2, or IDH1/2
  • Presence of 2 or more high-molecular-risk mutations
  • Hemoglobin level below 10 g/dL
  • Leukocyte count above 25 × 10 9/L
  • Platelet count below 100 × 10 9/L
  • Percentage of circulating blasts of 2% or higher
  • Bone marrow fibrosis grade 2 or higher
  • Constitutional symptoms

Life Expectancy According to GIPSS

GIPSS classifies patients with myelofibrosis into 4 risk categories:

  • Low (0 points), with a median survival of 26.2 years (94%)
  • Intermediate-1 risk (1 point), with a median survival of 8 years (73%)
  • Intermediate-2 risk (2 points), with a median survival of 4.2 years (40%)
  • High (3 or more points) with a median survival time of 2 years (14%)

The GIPSS is based exclusively on genetic risk factors:

  • VHR karyotype
  • Unfavorable karyotype
  • Absence of CALR type 1/like mutation
  • Presence of ASXL1SRSF2, and U2AF1 Q157 mutations

Some experts strongly suggest using GIPSS to predict patient prognosis and guide treatment decisions. Patients at low risk may be managed with long-term observation, whereas allogeneic stem cell transplant may be considered for transplant-age patients at high risk.

Life Expectancy Relative to Treatment Response

The only potentially curative treatment for myelofibrosis is HSCT. The cure rate with this treatment has been reported to be between 30% to 65%, but this comes at an increased mortality risk. Studies indicate that the 3- to 5-year survival rate after allogeneic stem cell transplant ranges from 45% to 70%; relapse and transplant-related mortality typically occur within the first 2 years after treatment.

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