How Long Will I Live With Myelofibrosis?

What is the Life Expectancy for Someone with 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:

1. Disease stage: Myelofibrosis has different stages, ranging from early to advanced stages. Patients with early-stage disease generally have a better prognosis than those with advanced-stage disease.
2. Age: Age can impact prognosis, with older patients potentially facing more complications and challenges in managing the disease. Most patients are diagnosed at approximately 65 years.
3. Symptoms and complications: Prognosis is influenced by the severity of symptoms and complications, such as an enlarged spleen or recurrent infections.
4. Genetic mutations: Specific genetic mutations, such as mutations in genes like JAK2, CALR, or MPL, can significantly impact prognosis by affecting disease progression and treatment response.
5. Other health conditions, like cardiovascular diseases, can complicate the management of myelofibrosis and impact overall health.
6. Response to treatment: 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.
7. Transformation to acute leukemia: In some cases, myelofibrosis can transform into acute leukemia, which typically has a poorer prognosis.

What Causes Death in Myelofibrosis?

The most common causes of death in patients with myelofibrosis include infection, bleeding, heart failure, mortality following spleen removal surgery, and myelofibrosis transforming into acute myeloid leukemia (AML). Other causes of mortality include kidney failure, liver failure, and blood clots. Most causes of death are disease or treatment-related; however, others are related to pre-existing conditions.

How Do Age and Sex Impact Prognosis? 

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. Patients aged 60 or younger at diagnosis generally have a less severe form of the disease, making them more likely to survive for over 20 years. However, patients diagnosed with intermediate or high-risk primary myelofibrosis will have a poorer prognosis (usually less than 5 years survival), regardless of age.

It has been observed a higher diagnosis of primary myelofibrosis in men than in women, with a 3:2 ratio. Amongst patients with better prognoses, women with primary myelofibrosis survived longer than men.  

How Does Prognosis Change When Patients Have Other Diseases?

Since myelofibrosis mainly impacts an older population, it is often accompanied by chronic health conditions, also referred to as comorbidities.

Some of these pre-existing conditions are linked with the most impactful effects on treatment selection and overall survival, including high blood pressure, high cholesterol, smoking, hepatitis C, diabetes, kidney disease, and lung disease. 

A recent study on patients with primary and secondary myelofibrosis examined the impact of common comorbidities on patient survival. The study showed that, in some cases, the presence of these comorbidities directly affected risk assessment and treatment selection. For example, it could mean that a patient may not be eligible for bone marrow transplantation, which is currently considered the only potential curative option.

The diseases that have a greater impact on reduced survival are kidney disease and other renal complications, severe infectious complications, and cardiopulmonary conditions such as pulmonary hypertension.

Primary and Secondary Myelofibrosis Have Different Prognosis?

The terms primary and secondary differentiate cases of myelofibrosis that develop on their own from those that originate from other blood cancers, such as polycythemia vera and essential thrombocythemia, both types of MPN.

The mortality risk of secondary myelofibrosis (sMF) is higher than in patients diagnosed with primary myelofibrosis (PMF). Polycythemia vera and essential thrombocythemia are generally considered to be less aggressive MPN than primary myelofibrosis. However, there are differences in genetic mutations between patients with secondary myelofibrosis that affect the disease progression.

Other risk factors can contribute to a poorer prognosis of primary myelofibrosis. For example, transformation into leukemia occurs in 10% to 20% of patients with primary myelofibrosis within the first 10 years. On the other hand, 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.

What Tools Can Help Predict the Risk and Survival of a Patient with Myelofibrosis?

Myelofibrosis can progress differently for each person. To help predict your individual outlook, doctors use risk stratification scores. These scoring systems combine various factors, including age, blood counts, symptoms, and genetic mutations, to estimate your risk of developing serious complications, the probability of relapse, needing further treatment, or your overall survival. While these scores are valuable tools, they have limitations, and it's important to work with your medical team to interpret them in the context of your own health journey.

In myelofibrosis, several scoring systems are used and continuously reviewed for accurate risk stratification of patients: the International Prognostic Scoring System (IPSS), 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).

Continue reading about each myelofibrosis risk score system: 

• Understanding Myelofibrosis Prognosis
• How is Myelofibrosis Staged and Classified?

How Does Treatment Response Impact Life Expectancy?

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.

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Sources:

• Myelofibrosis and Survival Prognostic Models: A Journey between Past and Future
• One Thousand Patients With Primary Myelofibrosis: The Mayo Clinic Experience
• 20+ Years and alive with primary myelofibrosis: Phenotypic signature of very long-lived patients
• Patient-specific comorbidities as prognostic variables for survival in myelofibrosis