This is a rare blood cancer type that begins in the marrow (a tissue inside the bones that produces blood cells). This cancer type is known as myelofibrosis and it negatively affects the blood cells the body creates.
Symptoms
This is a long-lasting condition that often worsens over time. However, some people live with myelofibrosis for years without any problems while others experience some symptoms that require treatment. Check below some myelofibrosis symptoms:
- Anemia (low levels of red blood cells) that causes fatigue, shortness of breath, and pale skin
- Neutropenia (reduced amount of white blood cells) that leads to frequent infections
- A lack of platelets in the blood (thrombocytopenia) that provokes easy bleeding and bruising
- Swollen spleen (splenomegaly) or liver (hepatomegaly)
- Night sweats
- Fever
- Itchy skin
- Joint or bone pain
- Weight loss
- Blood clots
- Increased blood pressure (hypertension)
- Esophagus or stomach bleeding
In case you notice any of the previous symptoms, immediately contact your healthcare professional.
Causes
The primary cause of myelofibrosis is genes that make stem cells not work properly. These stem cells produce blood cells in the bone marrow and if you get myelofibrosis, they become inflamed and appear scar tissue.
Approximately 90% of those with myelofibrosis experience one of the following gene changes. For example JAK2, CALR, or MPL. Moreover, healthcare professionals do not understand why these gene changes occur. Mostly, people do not get these genes from biological parents as well as you cannot pass them to your biological children.
The problem is that these genes multiply and spread through marrow and prevent your body from producing normal blood cells.
Unfortunately, it is not possible to prevent this condition.
Risk Factors
This cancer type happens mostly in older adults over 60 years old. Approximately 18,000 people live with myelofibrosis in the U.S. Teens and small children can get this condition in rare cases. However, when it occurs girls are twice as prone to develop it.
Additionally, myelofibrosis may occur itself or if it spreads to the marrow from other parts of the body. For instance, if you have myeloma or leukemia you are at higher risk of getting myelofibrosis. Furthermore, if you are exposed to toxic chemicals (such as Benzene) for long periods of time you may get this cancer type.
What Are The Effects of Myelofibrosis on The Body?
There are three types of blood cells that travel from bone marrow to the whole body. When a person gets this cancer type, their production is slowed. Check below them in detail:
- Red Blood Cells – These cells are responsible for supplying oxygen to organs and tissues (such as muscles). In case you have a low red blood cell count, the following symptoms may happen including bone pain, shortness of breath, weakness, fatigue, and lightheadedness.
- White Blood Cells – These cells are responsible for fighting against infections.
- Platelets – These help to clot the blood when you are cut. So it forms a scab and heal and if you do not have enough platelets, it may be difficult to stop bleeding.
Additionally, when the bone marrow does not produce blood cells, some organs (including the spleen, liver, or lungs) may begin to produce them. Moreover, the spinal cord and lymph nodes also can start making blood cells. As a result, organs that produce blood cells may become large and you may experience fullness and pain in the belly. If you experience this, immediately get medical help.
Diagnosis
There are no tests that help to diagnose myelofibrosis but a routine checkup may help before you experience any symptoms. In case the symptoms appear, your physician will ask questions about symptoms and medical history and will perform a physical examination to check for an enlarged spleen. They can also order you to do some tests. For example:
- Blood tests (including a complete blood count or CBC and a comprehensive metabolic panel)
- Imaging tests (including MRI and ultrasound)
- Gene tests
- Bone marrow tests (doctors will perform a biopsy)
Treatment
The treatment usually depends on the symptoms, the severity of the condition, and other factors. Approximately all treatments are focused on conditions that myelofibrosis provokes. For example:
Anemia
- Glucocorticoid medicines including Prednisone
- Human-made male hormones (androgens) such as Danazol
- Immunomodulators (medicines that affect the immune system) include Interferon alfa, Lenalidomide, or Thalidomide.
- Kinase inhibitors include Fedratinib, Pacritinib, Ruxolitinib, and others.
- Chemotherapy drugs (including Cladribine and Hydroxyurea)
- Blood transfusions
Swollen Spleen
- Hydroxyurea
- Interferon and others
In addition, Ruxolitinib is the first FDA-approved medicine in the treatment of intermediate or high-risk myelofibrosis. However, in severe myelofibrosis cases, you may need surgery that removes the spleen (splenectomy) or radiation therapy.
The only way to cure myelofibrosis is a transplant of stem cells or bone marrow from another healthy person. However, it may provoke certain serious side effects and physicians recommend this treatment option for people who do not have any other health problems. Discuss with your healthcare professional for treatment that is best for you.
Complications
Not everyone with myelofibrosis experience complications. Check below some of them:
- Belly and back pain
- Bleeding
- Joint and bone pain
- Growths
- Gout
- Hypertension
- Acute leukemia
Talk with a doctor right away if you get any of the complications listed above.
Frequently Asked Questions
Is it possible to cure myelofibrosis?
The only way it can be cured is a stem cell or bone marrow transplant. However, it is possible for people without any other medical conditions only. Otherwise, it may lead to certain dangerous adverse reactions.
What are the main causes of death in people with myelofibrosis?
These include infection, death after splenectomy, cardiac failure, hemorrhage, myeloid leukemia (AML), renal failure, hepatic failure, and thrombosis. Talk with your doctor for more details.
What is life expectancy in people with myelofibrosis?
Commonly, the life expectancy in people with this cancer type fluctuates around 6 years. It means that someone can live less than 6 years while other people live more than this rate. In case you have additional questions, ask your healthcare professional.
