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.
Myelofibrosis (MF) is a rare and serious form of bone marrow cancer. It affects the bone marrow’s typical function as fibrous tissue replaces healthy tissues. This impairs the marrow’s ability to produce sufficient red blood cells, white blood cells, and platelets.
Because it is a progressive disease, it worsens over time but affects each person differently. While some people have severe symptoms that progress quickly, others may live without symptoms for years.
Therefore, the MF outlook varies significantly.
Read on to learn more about myelofibrosis prognosis, including symptoms and management strategies.
Predicting the life expectancy of someone with MF is challenging. Unlike other cancers, doctors do not use a staging system to measure the severity of the condition.
Instead, they use an outlook scoring system to help them understand how MF will likely progress and the individual’s outlook.
The Dynamic International Prognostic Scoring System (DIPSS) Plus system looks at the following factors:
- whether someone’s age is over 65
- whole body symptoms, such as fever, fatigue, and weight loss
- anemia, or a low red blood cell count
- an atypically high white blood cell count
- circulating immature white blood cells, or blasts, greater than 1%
- need for transfusions
- specific chromosomal abnormalities
Each risk factor is a point. The total score indicates the following risk group:
- 0 points = low risk
- 1 point = intermediate-1 (INT-1) risk
- 2 or 3 points = intermediate-2 (INT-2) risk
- 4 or more points = high risk
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.
Given these variables, people should discuss their outlook and survival with a doctor.
MF may cause no symptoms for years. However, late stage MF can present with symptoms that include:
- Severe anemia: The extensive scarring in the bone marrow can lead to severe anemia. This can cause weakness, fatigue, and shortness of breath.
- Enlarged spleen and liver: These organs enlarge as they attempt to take over some of the job of making blood cells. This can cause abdominal pain and high blood pressure in certain veins, which doctors call portal hypertension.
- Weight loss: Late stage myelofibrosis may cause unintentional weight loss.
- Bone pain: Progressive bone marrow scarring can lead to bone pain, affecting mobility and quality of life.
Around 10–20% of people with MF go on to develop acute myeloid leukemia. This type of leukemia is difficult to treat and often has negative outcomes.
If someone is in the low risk category, a doctor may opt to observe their symptoms. For others, treatments for MF aim to alleviate symptoms and improve quality of life.
Treatment methods
- Drug therapy: Doctors prescribe medications, such as Jakafi (ruxolitinib), to control symptoms such as an enlarged spleen and bone pain. These targeted therapies may slow disease progression.
- Blood transfusions: Red blood cell transfusions can help manage anemia and associated fatigue.
- Splenectomy: In severe cases, surgically removing the spleen may help relieve discomfort.
- Allogenic stem cell transplantation: This is the only curative treatment for MF. It involves replacing a person’s atypical blood-forming stem cells with new ones from a donor.
Living with MF can be challenging. Therefore, individuals may find these strategies helpful:
- Find a specialist: For example, a person can look for a hematologist specializing in MF or myeloproliferative neoplasms for personalized care.
- Get educated: Individuals and loved ones can learn about MF to effectively advocate for themselves.
- Assess financial options: People can investigate reimbursement and financial aid programs for MF treatment.
- Organize paperwork: A person needs to maintain comprehensive medical records to allow for straightforward treatment management.
- Share and connect: Individuals can find support groups that help people connect with others living similar experiences.
When facing an MF diagnosis, consider discussing these questions with a healthcare professional:
- What are my treatment options, and what are the potential benefits and risks?
- How can we manage pain and discomfort effectively?
- Are there clinical trials or experimental treatments that I should consider?
- What is the expected progression of my condition, and what are the indicators of worsening symptoms?
- What supportive services or palliative care options are available to enhance my quality of life?
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.
Collaborating with a knowledgeable healthcare team can significantly optimize treatment, allowing people to access the most current and effective options.