Myelofibrosis - Medtick

Myelofibrosis

What is it?

An uncommon disease affecting the bone marrow which disrupts the body’s ability to produce healthy blood cells including red blood cell (helps to deliver oxygen to our organs and remove carbon dioxide), white blood cells (helps with our immune system) and platelets (helps with blood clotting).

  • Instead mutated blood cells are produced or poor quality.
  • It then causes the progressive scarring (fibrosis) of bone marrow (the spongy material inside some of your bones), leading to abnormal blood cell levels due to over activity by the bone marrow.
  • Quality blood cell production is reduced in the bone marrow  and then blood cell production then starts to take place in the liver and spleen instead. As the liver and spleen aren’t as good at producing blood cells, people with this condition may develop anaemia symptoms (not enough red blood cells in your blood).
  • The spleen may also become enlarged, as it ‘holds on’ to red blood cells instead of releasing them into the blood.

Cause

  • Mutations most closely linked myelofibrosis include the JAK2 (V617F) , CALR, or MPL gene (90% related)- (the stem cells start producing high levels of blood cells even when they’ve not been ‘told’ to do so by the growth factor (A growth factor is a substance which sends signals to your stem cells, so they can produce the right number of blood cells to keep you healthy).
  • Unknown (10%)
  • A family history of myeloproliferative disease
  • Jewish descent
  • Older age
  • Certain autoimmune condition:
  • Polycythaemia vera
  • Essential thrombocythemia (the body produces to much platelets)

Symptoms

  • General discomfort (muscle weakness), uneasiness or ill feeling (malaise) and/or fatigue (tiredness)?
  • Shortness of breath/breathing difficulties?
  • Pale appearance?
  • Bruise and bleed easily?
  • Unexplained weight loss?
  • Increased risk of chest infections?
  • Skin nodules?
  • Gout symptoms?
  • Sore Bone pain?
  • Lump appear on left hand side of abdomen due to the spleen swelling?
  • Swollen liver?
  • Leukaemia symptoms?
  • Anaemia symptoms?
  • Blood disorders symptoms?

Complications /Information to beware of/General tips:

Medical Emergency Condition

This condition can lead to:


This condition may show similar symptoms to:

  • Metastatic cancers (cancers that spread from other parts of the body to the bone marrow)
  • Polycythemia vera (a type of blood cancer that causes the overproduction of blood cells)
  • Hodgkin and non-Hodgkin lymphoma (cancers of the lymphatic system)
  • Multiple myeloma 
    • Rare type of blood cancer characterized by improper function and excessive production of plasma cells—a type of white blood cell—found in bone marrow.
    • The immune system is impacted due to the inefficiency of the plasma cells to produce healthy antibodies, which fight infection.
    • The overgrowth of plasma cells crowds other blood cells, reducing the number of healthy red and white blood cells which are involved in processes to give us energy and fight infection .
    • Common symptoms include leukaemia symptoms and anaemia symptoms in particular bone pain, usually experienced in the lower back and ribs and if not treated it can lead to weak bones, destruction of bones as well as a build up of abnormal antibodies called ‘M’ Proteins which damage the kidneys  and  kidney stones due to increase production of uric acid as well as blood disorders symptoms.
  • Acute leukaemia (a blood cancer affecting leukocytes)
  • Chronic myeloid leukaemia (cancer of the bone marrow)
  • Exposure to certain chemicals, such as benzene or petroleum
  • Radiation exposure
  • Gaucher disease

Please talk to your healthcare professional (i.e. Medical Doctor/Pharmacist) for further advice

Detailed Information

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