Life Expectancy in Myelofibrosis
Myelofibrosis is a rare type of blood cancer that starts in the bone marrow. It is considered a type of chronic leukemia. It can occur on its own (primary myelofibrosis) or due to an illness (secondary myelofibrosis).
When you have myelofibrosis, the following questions may come to your mind:
- What is the myelofibrosis life expectancy rate?
- Is there a myelofibrosis life expectancy with treatment?
- Can I survive myelofibrosis?
- Is myelofibrosis a terminal illness?
Before answering these questions, it is necessary to understand myelofibrosis well.
What is Myelofibrosis?
Myelofibrosis (MF) is a rare type of bone marrow cancer that disrupts the body’s average blood cell production. It is also considered chronic leukemia because it affects the blood-forming tissues in the body.
“Myelo” means bone marrow. The bone marrow produces immature blood cells that can develop into specialized blood cells such as red blood cells, white blood cells, or platelets and give them to the blood. As a result, all the blood cells necessary for the body are produced healthily.
Myelofibrosis occurs when the average production of these blood cells is disrupted. The scar tissue accumulates in the bone marrow, blood cells cannot develop properly, and normal bone marrow tissue is gradually replaced by an abnormal tissue called fibrosis. A change (mutation) in the DNA of a single cell will be transferred to new cells when the mutated cell divides.
People generally have no symptoms when they are first diagnosed. The most common myelofibrosis symptom is pain and swelling in the spleen, followed by symptoms of anemia. It can include unexplained tiredness, weakness, fever, increased sweating, especially at night, shortness of breath, and palpitations.
Some specific gene mutations have been identified in people with myelofibrosis. The most common is the Janus kinase 2 (JAK2) gene. People who are thought to have MF are screened for this gene. The mutation in the JAK2 gene and its relationship with myelofibrosis are essential in determining the course of the disease and the type of treatment.
Myelofibrosis can affect anyone, but some factors are known to increase the illness risk:
- Age (It is most often diagnosed in people older than 50).
- Another blood cell disorder.
- Exposure to certain chemicals.
- Radiation. (People exposed to very high radiation levels have an increased risk of myelofibrosis.)
Stages of Myelofibrosis
Myelofibrosis usually develops slowly, and primary MF doesn’t have clearly defined stages. The doctors may categorize the illness into a low-, intermediate-or high-risk group. Many people don’t experience signs or symptoms in their very early stages.
In order to determine the stages of the disease, it is vital to know the form of the disease. Although the exact cause of mutations in the bone marrow is unknown, the disease occurs in two ways. Firstly, people who have no history of problems with their bone marrow can get MF. It is called primary myelofibrosis (PMF) or chronic idiopathic myelofibrosis and agnogenic myeloid metaplasia. Secondly, myelofibrosis can develop from another bone marrow disorder, and it is called secondary myelofibrosis.
Prognosis for Myelofibrosis
There is no staging system in myelofibrosis. However, doctors and researchers use factors defined in the international prognosis scoring system (IPSS), which can help them estimate patients’ average years of survival.
The natural course of the disease can vary significantly depending on the occurrence of the disease. The disease remains stable for long periods in some patients, and they lead lives with easy treatment. The disease progresses very rapidly in some patients, and myelofibrosis treatment methods are applied to help alleviate myelofibrosis symptoms. Conversion to acute myeloid leukemia occurs in 10 to 20 percent of cases.
Is Myelofibrosis a Terminal Illness?
Various methods are available for treating myelofibrosis, depending on the patient’s symptoms and individual circumstances. In order to say whether the disease is terminal or not, it is necessary to consider conditions such as genetic risk factors, age, sex, blood count and determine the stages of the disease. Some people may not exhibit symptoms for years.
In addition, clinical studies for myelofibrosis patients in risk groups continue, and different treatment methods are being tried. In the treatment of myelofibrosis, techniques such as antibiotic therapy, biotherapy, radiotherapy, chemotherapy, stem cell transplantation can be applied, and successful results have been measured with these methods.
The Survival Rate of Myelofibrosis
The median survival of patients with primary myelofibrosis is approximately 6 years. It has been observed that the natural course of the disease has changed with studies such as allogeneic hematopoietic stem cell transplantation (allo-HSCT). Of the 1,282 patients identified in the Mayo Clinic database between 1976 and 2017, 26 percent lived 20+ years, but 49 percent died within the first 5 years following diagnosis.
According to a study published in Haematologica, in total, 2459 patients were identified through the Human Mortality Database who had received a first HSCT (Hematopoietic Stem Cell Transplant) between January 1995 and December 2014 for primary or secondary MF. Of this, 1055 patients had been reported alive and disease-free two years after HSCT.