Myelofibrosis prognosis depends on many factors and is different for each patient. Doctors can determine the overall prognosis after testing is completed for the patient. The International Prognostic Scoring System (IPSS) weighs important individual factors to calculate the severity of the disease and how long the patient may survive after diagnosis.
How is myelofibrosis prognosis determined? Because myelofibrosis has a heterogeneous presentation, determining a patient’s prognosis can be difficult. Myelofibrosis is a rare blood cancer that disrupts the production of blood cells and eventually causes scarring of the bone marrow. Myelofibrosis belongs to a group of chronic blood disorders called myeloproliferative neoplasms (MPNs). Primary myelofibrosis occurs when there are no MPNs or autoimmune diseases present prior to diagnosis. Secondary myelofibrosis occurs when there is a previous MPN such as polycythemia vera, essential thrombocythemia, or others.
The IPSS is used to dictate the myelofibrosis prognosis in individual patients. This system also helps determine the overall prognosis including the estimated years of survival. The factors that affect the IPSS outcome include:
- Age over 65
- Symptoms experienced include weight loss, fatigue, and fever
- If anemia is present from lack of red blood cells
- White blood cell count
- Circulating blood blasts greater than 1 percent, which are immature white blood cells
The outcomes of the IPSS are:
- Low risk: None of the factors are applicable and the expected survival is an estimated 10 years or longer
- Intermediate risk: One of the factors is met, the expected survival is an estimated 8 years.
- High risk: 3 or more factors are met; the expected survival is 2 years
You will also be considered for genetic testing to identify any abnormalities or mutations present. Mutations are often found in the JAK2 in more than half of myelofibrosis cases, as well as the CALR, MPL, and TET2 genes. Identifying a gene mutation will also factor into the overall prognosis as well as influence the treatment decision.
There is an additional test that can be used after the diagnosis is confirmed, anytime throughout the course of the disease. This test is called the Dynamic International Prognostic Scoring System (DIPSS) and although the factors are the same, the weight of each factor is different.
The symptoms that are experienced from myelofibrosis have a direct impact on the treatment decision and overall prognosis for patients. Those who do not experience any symptoms will often opt for active surveillance or watchful waiting. This method waits until symptoms are experienced to begin treatment because the side effect of treatment my cause more harmful symptoms than the disease. The more symptomatic the patient is, the more aggressive the treatment will be. For higher risk patients, they may experience the following symptoms:
- Fatigue, weight loss, fever (considered in the IPSS score)
- Shortness of breath
- Easy bruising and bleeding
- Night sweats
- Loss of appetite or fullness