Myelofibrosis Prognosis

In recent years there have been several breakthroughs in the treatment of myelofibrosis. The myelofibrosis prognosis has drastically improved from new medications to entirely different treatment plans.

With so many new treatment options, predicting the course of myelofibrosis, otherwise known as the prognosis, becomes difficult. An accurate prognosis relies on the outcomes of individuals who have gotten the disease. However, in those historical outcomes, patients did not have access to novel medicines in treatments today. Therefore, the medical world is still learning how new Myelofibrosis treatments impact the prognosis.

Myelofibrosis Prognosis Scoring System

When diagnosing myelofibrosis, it is common for a doctor to use the International Prognosis Scoring System (IPSS) to assess the severity of the disease. Some of the factors used in the IPSS include:

• Being over the age of 65
• The presence of constitutional symptoms (symptoms that affect multiple body systems like fevers, weight loss, fatigue, etc.)
• Having anemia (a low red blood cell count)
• Having an extremely high white blood cell count
• Circulating blood blasts greater than 1 percent
• Type of genetic mutation (JAK2, CALR, etc.)

As a general rule, the more factors a patient possesses, the greater the risk of the disease. Therefore, if several risk factors present, your doctor may take a more aggressive treatment approach. 

So with myelofibrosis, in particular, I am not in favor of such an approach because life is different and therapies are different right now. That’s why I apply that prognostic wisdom for only one purpose: to refer the patients to the bone marrow transplant.

Dr. Srdan Verstovsek | Read more about Dr. Verstovsek’s prognostic approach.

Myelofibrosis Life Expectancy

It’s essential to remember that life expectancy for patients with myelofibrosis has changed significantly over the past five to ten years. Unfortunately, the improvements in treatment options have not been around long enough to substantially alter the historical median survival rate of patients with MF.

The median life expectancy for a patient with myelofibrosis is six years from the initial diagnosis. However, from 1976 through 2017, 26% of patients diagnosed with MF have lived 20+ years. Additionally, myelofibrosis is most commonly diagnosed in people over the age of 65, which can also impact life expectancy.

According to the IPSS scoring system, the median survival rate based on the number of risk factors varies significantly.

While the prognostic criteria can help determine life expectancy, it’s more important to help patients get the treatment they need to live for as long as possible. For example, if a patient’s life expectancy is less than five years, a doctor may recommend a bone marrow transplant.

Bone marrow transplants are currently the only potential cure for myelofibrosis. The procedure comes with extreme risks, but the prognostic criteria give patients the information they need to make the best decision for them.

The Progression of Myelofibrosis

If you are diagnosed with myelofibrosis or caring for an individual with the disease knowing the disease progression is paramount to understanding when the risk of the disease increases. MF progresses in four stages:

• Stage 1: overactive signaling leads to the proliferation of malignant stem cells. Most patients don’t experience any symptoms during this phase.
• Stage 2: an increased production of abnormal megakaryocytes occurs.
• Stage 3: increased production of inflammatory cytokines and myofibroblasts
• Stage 4: Extensive fibrosis (scarring) in the bone tissue and failure of the bone marrow leading to reduced blood cell production.

Treatments and symptoms will vary from stage to stage. The treatments at each stage are geared toward preventing the progression of the disease and the symptoms associated with each stage.

But in the aftermath of that, that grief was so heavy. That grief was harder than the grief of getting cancer.

Mary L. | Read more about Mary’s emotional grief with her MF diagnosis.

Coping with Myelofibrosis

Receiving a myelofibrosis diagnosis is extremely stressful and life-altering. If you receive a diagnosis, we encourage you to develop a strong support structure, including your doctor, family, friends, and a mental health professional, to help you work through these life changes. For resources on managing a cancer diagnosis and treatment, visit our Navigating Cancer page.