Definition

What is myelofibrosis?

Myelofibrosis is a serious bone marrow disorder that disrupts your body’s normal production of blood cells. The result is extensive scarring in your bone marrow, leading to severe anemia, weakness, fatigue and often an enlarged spleen.

Myelofibrosis is an uncommon type of chronic leukemia — a cancer that affects the blood-forming tissues in the body. Myelofibrosis belongs to a group of diseases called myeloproliferative disorders.

Many people with myelofibrosis get progressively worse, and some may eventually develop a more serious form of leukemia. Yet it’s also possible to have myelofibrosis and live symptom-free for years. Treatment for myelofibrosis, which focuses on relieving symptoms, can involve a variety of options.

How common is myelofibrosis?

Myelofibrosis is uncommon and affects about 1 in 20,000 people. Myelofibrosis usually affects middle-aged and elderly people but can occur at any age. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of myelofibrosis?

The common symptoms of myelofibrosis are:

  • Feeling tired, weak or short of breath, usually because of anemia
  • Pain or fullness below your ribs on the left side, due to an enlarged spleen
  • Easy bruising
  • Easy bleeding
  • Excessive sweating during sleep (night sweats)
  • Fever
  • Bone pain

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes myelofibrosis?

Myelofibrosis occurs when blood stem cells develop a genetic mutation. Blood stem cells have the ability to replicate and divide into the multiple specialized cells that make up your blood — red blood cells, white blood cells and platelets.

It’s not clear what causes the genetic mutation in blood stem cells.

As the mutated blood stem cells replicate and divide, they pass along the mutation to the new cells. As more and more of these mutated cells are created, they begin to have serious effects on blood production.

The end result is usually a lack of red blood cells — which causes the anemia characteristic of myelofibrosis — and an overabundance of white blood cells with varying levels of platelets. In people with myelofibrosis, the normally spongy bone marrow becomes scarred.

Several specific gene mutations have been identified in people with myelofibrosis. The most common is the Janus kinase 2 (JAK2) gene. Knowing whether the JAK2 gene or others are associated with your myelofibrosis helps determine your prognosis and your treatment.

Risk factors

What increases my risk for myelofibrosis?

There are many risk factors for myelofibrosis, such as:

  • Myelofibrosis can affect anyone, but it’s most often diagnosed in people older than 50.
  • Another blood cell disorder. A small portion of people with myelofibrosis develop the condition as a complication of essential thrombocythemia or polycythemia vera.
  • Exposure to certain chemicals. Myelofibrosis has been linked to exposure to industrial chemicals such as toluene and benzene.
  • Exposure to radiation. People exposed to high levels of radiation, such as survivors of atomic bomb attacks, have an increased risk of myelofibrosis. Some people who received a radioactive contrast material called Thorotrast, used until the 1950s, have developed myelofibrosis.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is myelofibrosis diagnosed?

Your doctor will initially arrange some blood tests to check for anaemia and also to check your white cell and platelet counts. If your doctor has any concerns that you might have myelofibrosis then you will be referred to a specialist in blood diseases (called a haematologist) for further investigations. These further investigations will usually include X-rays, magnetic resonance imaging (MRI) and also a bone marrow biopsy and aspiration (which can confirm the diagnosis of myelofibrosis).

How is myelofibrosis treated?

Immediate treatment may not be necessary if you do not have any symptoms and do not have anaemia or any other complications such as an enlarged spleen. Instead, your doctor is likely to monitor your health closely through regular check-ups. This will make sure you receive treatment if and when you need it. Some people remain without symptoms for many years.

If or when you do need treatment, this will include medicines to control the abnormal bone marrow cells. Medicines, such as hydroxycarbamide, can be used to control the abnormal bone marrow cells. Treatment will also be needed for the effects of reduced production of blood cells such as anaemia.

Thalidomide and other similar medicines (lenalidomide and pomalidomide) may also help to improve blood cell production and reduce the size of an enlarged spleen. These drugs may be used together with steroid medicines.

Treatments for anaemia

If myelofibrosis is causing severe anaemia then you may need regular blood transfusions. Taking a a medicine similar to the male hormone (androgen) may help your body to make red blood cells and so improve anaemia.

Treatments for an enlarged spleen

If an enlarged spleen is causing any complications, you may need surgical removal of your spleen (splenectomy). Splenectomy does have some risks, including infections, excessive bleeding and blood clot formation leading to stroke or pulmonary embolism. Chemotherapy or radiotherapy can also be used to reduce the size of the spleen, when surgical removal isn’t an option.

Stem cell transplant

Stem cell transplant from a suitable donor (allogeneic stem cell transplantation) is the only treatment that has the potential to cure myelofibrosis. Stem cell transplant is also called a bone marrow transplant. But it also has a high risk of life-threatening side-effects. Most people with myelofibrosis, because of age, stability of the disease or other health problems, don’t qualify for this treatment.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage myelofibrosis?

The following lifestyles and home remedies might help you cope with myelofibrosis:

  • Learn about your condition. Myelofibrosis is fairly uncommon. To help you find accurate and trustworthy information, ask your doctor to direct you toward appropriate sources. Based on these sources, find out as much as you can about myelofibrosis.
  • Get support. Take this opportunity to lean on family and friends. It can be tough to talk about your diagnosis, and you’ll likely get a range of reactions when you share the news. But talking about your diagnosis and passing along information about your condition can help. So can the offers of help that often result.

You may also benefit from joining a support group, either in your community or on the internet. A support group of people with the same or a similar diagnosis, such as a myeloproliferative disorder or another rare disease, can be a source of useful information, practical tips and encouragement.

  • Explore ways to cope with the disease. If you have myelofibrosis, you may face frequent blood work and medical appointments and regular bone marrow exams. Some days, you may feel sick even if you don’t look sick. And some days, you may just be sick of being sick.
  • Try to find some activities that help, whether it’s yoga, exercise, social outings or adopting a more flexible work schedule. Talk to a counselor, therapist or oncology social worker if you need help dealing with the emotional challenges of this disease.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: September 8, 2017 | Last Modified: September 8, 2017

Want to live your best life?
Get the Hello Doktor Daily newsletter for health tips, wellness updates and more.