Myelofibrosis can cause complications, such as extramedullary hematopoiesis, severe anemia, and acute myeloid leukemia, among others. Treatment may reduce risk.
Myelofibrosis (MF) is a chronic form of blood cancer where scar tissue in the bone marrow slows the production of healthy blood cells. A shortage of blood cells causes many symptoms and complications of MF, such as fatigue, easy bruising, fever, and bone or joint pain.
Many people don’t experience any symptoms in the early stages of the disease. As the disease progresses, symptoms and complications tied to abnormal blood cell counts may appear.
A doctor can help proactively treat MF as soon as you begin experiencing symptoms. Treatment can help reduce your risk of complications and increase survival.
Here’s a closer look at the potential complications of MF and how you can reduce your risk.
Your spleen helps fight infections and filters out old or damaged blood cells. It also stores red blood cells and platelets that help your blood clot.
When you have MF, your bone marrow can’t make enough blood cells due to scarring. Blood cells are eventually produced outside the bone marrow in other parts of your body, such as your spleen.
This is referred to as extramedullary hematopoiesis. The spleen sometimes becomes abnormally large as it works harder to make these cells.
An enlarged spleen (splenomegaly) can cause uncomfortable symptoms. It can cause abdominal pain when it pushes against other organs and makes you feel full even when you haven’t eaten much.
You can also develop extramedullary hematopoiesis in other organs, such as the liver.
When blood cells are produced outside the bone marrow, noncancerous tumors of developing blood cells can form in other areas of the body.
These tumors may cause bleeding inside your gastrointestinal system. This may make you cough or spit up blood. Tumors may also compress your spinal cord or cause seizures.
Blood flows from the spleen to the liver through the portal vein. Increased blood flow to an enlarged spleen in MF can cause high blood pressure in the portal vein.
An increase in blood pressure sometimes forces excess blood into the blood supply of the stomach and esophagus. This may rupture smaller veins and cause bleeding.
Platelets in the blood help your blood to clot after an injury. Platelet count can fall below typical ranges as MF progresses. A low number of platelets is known as thrombocytopenia.
Without enough platelets, your blood can’t clot properly. This can make you bleed more easily.
MF can harden your bone marrow. It can also lead to inflammation in the connective tissues around the bones. This leads to bone and joint pain.
MF causes the body to produce more waste products from increased blood cell turnover (blood cell destruction), such as uric acid. Levels of uric acid can accumulate above the typical amount. If the uric acid crystallizes, it sometimes settles in the joints. This is referred to as gout. Gout can cause swollen and painful joints.
A low red blood cell count, known as anemia, is a common MF symptom. Sometimes, anemia becomes severe and causes debilitating fatigue, bruising, and other symptoms.
For about 15 to 20% of people, MF progresses to a more severe form of cancer known as acute myeloid leukemia (AML). AML is a rapidly progressing cancer of the blood and bone marrow.
Your doctor may prescribe various treatments to address MF complications. These include:
- JAK inhibitors, including ruxolitinib (Jakafi) and fedratinib (Inrebic)
- immunomodulatory drugs, such as thalidomide (Thalomid), lenalidomide (Revlimid), interferons, and pomalidomide (Pomalyst)
- corticosteroids, such as prednisone
- surgical removal of the spleen (splenectomy)
- androgen therapy
- chemotherapy drugs, such as hydroxyurea
It’s essential to work with a doctor to manage MF. Frequent monitoring can help reduce your risk of MF complications. The doctor may request that you visit regularly for blood counts, other testing, and physical exams.
If you currently have no symptoms and low risk MF, there’s no evidence that you’ll benefit from earlier interventions. The doctor may wait to begin treatments until your condition progresses.
The doctor may prescribe treatments if you have symptoms or intermediate- or high risk MF.
The JAK inhibitors ruxolitinib (Jakafi) and fedratinib (Inrebic) target abnormal pathway signaling caused by a common MF gene mutation. These drugs have been shown to significantly reduce spleen size and address other debilitating symptoms, including bone and joint pain. Research suggests they may significantly reduce the risk of complications and increase survival.
Stem cell transplantation
An allogeneic stem cell transplantation is the only treatment that can potentially cure MF. It involves receiving an infusion of stem cells from a healthy donor, which replaces the faulty stem cells causing MF symptoms.
This procedure carries significant and potentially life threatening risks. Doctors typically only recommend it for people with MF with an expected survival of less than 5 years.
New MF treatments are constantly being developed. Try to stay up to date on the latest research in MF, and ask your doctor whether you should consider enrolling in a clinical trial.
What are the signs that myelofibrosis is progressing?
A doctor can monitor the progression of myelofibrosis through blood counts and physical exams. A low red blood or platelet count may indicate that myelofibrosis is progressing. You may also experience other symptoms, such as fatigue, shortness of breath, and abdominal pain due to an enlarged spleen.
What are the worsening symptoms of myelofibrosis?
Worsening myelofibrosis can cause fatigue, shortness of breath, gout, abdominal pain, anemia, and other symptoms.
What are the last stages of myelofibrosis?
Medications and blood transfusions may become less effective during the later stages of myelofibrosis. People may experience anemia and other complications. Some people with myelofibrosis may be able to get a bone marrow transplant.
What causes death with myelofibrosis?
People with myelofibrosis may experience fatal infections or hemorrhages due to low blood cell and platelet counts. Some procedures, such as a splenectomy or a bone marrow transplant, carry a risk of death. Myelofibrosis can also result in heart failure or acute myeloid leukemia, which can be fatal.
Myelofibrosis is a rare cancer where scarring keeps your bone marrow from producing enough healthy blood cells. If you have intermediate or high risk MF, several treatments can address symptoms, reduce your risk of complications, and potentially increase survival.
Many ongoing trials continue to explore new treatments. Stay in touch with the doctor treating your myelofibrosis and discuss the appropriate treatments.