Myelofibrosis

Medically Reviewed by Dr. K on 15 March 2021

What is Myelofibrosis

Myelofibrosis is a rare form of bone marrow cancer that interferes with the body's regular blood cell formation. Myelofibrosis is a type of chronic leukaemia, a cancer that affects the body's blood-forming tissues. Myelofibrosis is part of the myeloproliferative disorders category of diseases.

There are three categories of blood cells. They make their way from your bone marrow to the rest of the body. Each has a distinct role to play. They are unable to function normally in myelofibrosis.

  • Your organs and tissues, such as your muscles, receive oxygen from red blood cells. You can feel sluggish, out of breath, lightheaded, or very exhausted if you have too little. It's possible that you develop bone pain.
  • White blood cells assist in the fight against infection. If you produce too many, your body won't be able to protect you from sickness as well as it can.
  • When you have a wound, platelets help the blood to clot so you can recover by forming a scab. It can be difficult to avoid bleeding if you don't have enough functioning platelets.

Since your marrow has trouble producing blood cells, other organs such as your spleen, liver, or lungs can take over. Blood cells can also be generated in the spinal cord and lymph nodes (which are small glands located in the groin, neck, and armpits).

Organs, especially the spleen, may become too big as a result of all that extra blood. If this occurs, you can experience discomfort or a sensation of fullness in your abdomen. This may be dangerous, so you should get medical attention right away.

Some patients with myelofibrosis have no symptoms and may not need immediate care. Others with more severe types of the disease may require immediate care.

 

Symptoms

Myelofibrosis is a slow-progressing disease. Most patients do not exhibit any signs or symptoms when cancer is in its early stages.

Signs and signs of a disturbance of natural blood cell development include:

  • 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

Treatment

Myelofibrosis treatment, which focuses on symptom relief, may provide a range of options. Your treatment will be determined by your specific conditions, and your symptoms. If you don't have any, your doctor can advise you to wait to see what happens.

The majority of treatments target the complications of myelofibrosis. If you have anaemia, your doctor can suggest the following to you:

  • Glucocorticoid medications: prednisone
  • Human-made male hormones (androgens) like danazol
  • Drugs that affect your immune system (immunomodulators) such as interferon alfa, lenalidomide, or thalidomide
  • Chemotherapy drugs like cladribine and hydroxyurea
  • Blood transfusions

If your spleen is swollen, you may take:

  • Hydroxyurea
  • Interferon
  • Ruxolitinib

In extreme circumstances, you can require a splenectomy (surgery to remove your spleen) or radiation treatment.

The only treatment that could be able to reverse myelofibrosis is an allogeneic donation of stem cells or bone marrow from another human. It replaces the diseased cells in your bone marrow with healthy ones. However, since it may have adverse side effects, doctors only prescribe it to patients who are younger and have no other health issues. Consult a doctor on the best treatment option for you.

See your doctor on a daily basis and have your blood checked for any issues. Acute myeloid leukaemia, a form of cancer that is more difficult to treat, affects approximately 20% of people with myelofibrosis.

It's not easy to get a cancer diagnosis. It can be beneficial to speak with someone who understands what you're going through.

Sources

Referenced on 28/4/2021

  1. Leukemia and Lymphoma Society: “Myelofibrosis.”
  2. Myeloproliferative Research Foundation: “Primary Myelofibrosis.”
  3. National Organization for Rare Disorders: “Primary Myelofibrosis.”
  4. Expert Opinion on Pharmacotherapy: “Myelofibrosis: an update on drug therapy in 2016.”
  5. https://www.mayoclinic.org/diseases-conditions/myelofibrosis/symptoms-causes/syc-20355057
  6. https://www.webmd.com/cancer/myelofibrosis-causes-risk-factors

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