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Myelofibrosis: A Bone Marrow Disorder That Thickens With Scar Tissue
By Dr. Gajendra Kumar Himanshu
Myelofibrosis is a rare blood and bone marrow disorder in which abnormal blood-forming cells cause scar-like tissue, called fibrosis, to build up inside the bone marrow. As the marrow becomes less able to make healthy blood cells, a person may develop anaemia, tiredness, easy bruising or bleeding, and an enlarged spleen. Myelofibrosis can develop on its own, called primary myelofibrosis, or after another blood disorder such as polycythemia vera or essential thrombocythemia.
Myelofibrosis can affect people differently. Some people have no symptoms at first and discover the condition during a routine blood test, while others may have tiredness, weight loss, night sweats, or discomfort under the left ribs.
In this blog, we will explain what myelofibrosis is, why scar tissue develops in the bone marrow, its symptoms, diagnosis, treatment options, and when a stem cell transplant may be considered.
What Is Myelofibrosis?
Bone marrow is the soft tissue inside many bones that makes red blood cells, white blood cells, and platelets. These cells are important for carrying oxygen, fighting infections, and helping the blood clot.
In myelofibrosis, abnormal blood-forming cells cause too much fibrous tissue to build up in the bone marrow. This scar-like tissue makes it harder for the marrow to produce healthy blood cells. When this happens, the body may try to make blood cells in other organs, especially the spleen and liver. This can cause the spleen to become enlarged.
Myelofibrosis belongs to a group of conditions called myeloproliferative neoplasms (MPNs). These disorders cause abnormal production of blood cells in the bone marrow.
For a simple overview of blood-related cancers and disorders, you can also read our blog on the types of blood cancer.
What Causes Myelofibrosis?
The exact reason why myelofibrosis develops is not always known. In many patients, changes are found in genes such as JAK2, CALR, or MPL.
These changes can cause blood-forming cells to behave abnormally. Over time, this abnormal activity can lead to fibrosis in the bone marrow. However, having a gene change does not mean that a person inherited the condition from a parent. Many of these changes develop during a person’s lifetime.
Myelofibrosis may occur as a primary condition or develop after another myeloproliferative disorder. When it develops after another condition, it is sometimes called secondary myelofibrosis.
What Are the Symptoms of Myelofibrosis?
Some people have no symptoms in the early stages. The condition may be found when a routine blood test shows an unusual blood count.
As the disease progresses, symptoms may develop because the bone marrow is not making enough healthy blood cells or because the spleen becomes enlarged.
Common symptoms can include:
- Persistent tiredness or weakness
- Shortness of breath
- Pain or fullness under the left ribs
- Feeling full after eating only a small amount
- Easy bruising or bleeding
- Bone pain
- Fever
- Night sweats
- Unexplained weight loss
An enlarged spleen can press on the stomach, making a person feel full quickly even after eating a small meal.
These symptoms can have many other causes, so they do not automatically mean someone has myelofibrosis. However, symptoms that continue or worsen should be evaluated by a doctor.
You can also read our blog on blood cancer symptoms to understand when changes in blood counts or unexplained symptoms may need medical attention.
How Does Myelofibrosis Affect Blood Cells?
As scar tissue builds up in the bone marrow, the marrow may produce fewer healthy blood cells. This can affect all three major blood cell types.
Red Blood Cells
A low red blood cell count can cause anaemia. This may lead to tiredness, weakness, dizziness, or shortness of breath.
White Blood Cells
White blood cells help fight infections. Changes in their number or function can affect the body’s ability to respond to infections.
Platelets
Platelets help the blood clot. Myelofibrosis can cause either low or high platelet counts, depending on the stage and individual disease pattern. Abnormal platelet levels can increase the risk of bleeding or blood clots.
Myelofibrosis Symptoms and Effects at a Glance
The table below summarises the main problems myelofibrosis can cause and what each one may lead to.
Problem | What may happen |
|---|---|
Bone marrow fibrosis | Scar-like tissue builds up in the marrow |
Anaemia | Tiredness, weakness, or shortness of breath |
Enlarged spleen | Fullness or pain under the left ribs |
Low platelets | Easy bruising or bleeding |
Abnormal white blood cells | Increased risk of infections in some patients |
Bone changes | Bone or joint pain may occur |
Blood formation outside marrow | Liver or spleen may start making blood cells |
The severity of these problems varies from person to person.
How Is Myelofibrosis Diagnosed?
Doctors usually use several tests to diagnose myelofibrosis. There is no single blood test that can confirm every case.
A complete blood count (CBC) can show changes in red blood cells, white blood cells, platelets, and haemoglobin. Doctors may also examine a blood sample under a microscope to look for unusual blood cells.
A bone marrow biopsy is an important part of diagnosis. During this test, a small sample of bone marrow is removed and examined to look for fibrosis and abnormal blood-forming cells. Genetic tests may also be performed to look for changes such as JAK2, CALR, or MPL.
Doctors may also use imaging tests such as an ultrasound or CT scan to check whether the spleen or liver is enlarged.
How Is Myelofibrosis Treated?
Treatment depends on symptoms, blood counts, age, overall health, disease risk, and other factors. Not everyone needs immediate treatment. Some people with few or no symptoms may be monitored regularly through blood tests and medical checkups.
When treatment is needed, doctors may use medicines to control symptoms and reduce complications. Targeted medicines such as ruxolitinib may help reduce an enlarged spleen and some symptoms in appropriate patients. Treatment for anaemia may include blood transfusions or medicines that support red blood cell production in selected cases.
Other treatments may be considered depending on the patient’s condition.
Can a Stem Cell Transplant Treat Myelofibrosis?
An allogeneic stem cell transplant is currently the main treatment with the potential to provide long-term disease control or cure for some patients with higher-risk myelofibrosis. It uses blood-forming stem cells from a donor.
However, a transplant is a major treatment and is not suitable for everyone. Doctors consider factors such as age, overall health, disease risk, symptoms, donor availability, and the potential benefits and risks before recommending it.
Living With Myelofibrosis
Myelofibrosis is often a long-term condition, so regular medical follow-up is important. Blood tests help doctors monitor blood counts and changes in the disease. Your doctor may also monitor the size of the spleen and changes in symptoms.
People with myelofibrosis should tell their healthcare team about new or worsening tiredness, bleeding, fever, increasing abdominal discomfort, unexplained weight loss, or other changes.
Treatment can also change over time. A plan that works well at one stage may need to be adjusted if symptoms or blood counts change.
If you want to read more about myeloproliferative neoplasms treatment, you can visit the National Cancer Institute.
Consult Today
Myelofibrosis is a complex bone marrow disorder that can gradually affect the body’s ability to make healthy blood cells. Although it may not cause symptoms at first, ongoing tiredness, unexplained weight loss, night sweats, unusual blood counts, or fullness under the left ribs should be medically evaluated.
At Oncare Cancer Hospital, cancer and blood disorder specialists can review blood tests, bone marrow biopsy results, genetic findings, and other medical information to understand the condition and discuss suitable treatment options. Depending on the patient’s needs, treatment may include monitoring, medicines, supportive care, or stem cell transplantation.
If you or a family member has been diagnosed with myelofibrosis, an individual treatment plan is important because the condition can behave differently in each person.
Disclaimer
This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always discuss decisions about blood tests, medicines, or any treatment for a bone marrow disorder with a certified cancer healthcare provider.
Frequently Asked Questions
Myelofibrosis is a chronic blood and bone marrow disorder classified as a myeloproliferative neoplasm. It can behave like a blood cancer and requires specialist monitoring.
The bone marrow develops scar-like fibrous tissue, making it harder to produce healthy blood cells.
A donor stem cell transplant may offer the possibility of long-term disease control or cure for some suitable higher-risk patients, but it is not appropriate for everyone.
When the bone marrow cannot make enough blood cells, the body may start making blood cells in the spleen and liver. This can cause the spleen to become enlarged.
Written and Verified by:
Dr. Gajendra Kumar Himanshu Exp: 10 Yr
Medical Officer
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