Agnogenic myeloid metaplasia

 

Do you know? All your blood cells are produced in bone marrow. What if the bone marrow gets diseased? Then, the normal production of blood cells is drastically disrupted.

Agnogenic myeloid metaplasia, also called myelofibrosis is one such disease that affects the production of blood cells. Your bone marrow is replaced with fibrous scar tissue leading to severe anemia, fatigue, enlargement of spleen and liver. It is an uncommon type of chronic leukemia, which means cancer that affects the blood-forming cells. Most of the people may survive with symptom-free periods for years but in few, the condition would worsen up to leukemia. The treatment purely focuses on the improvement of the quality of life by relieving the symptoms.

The exact cause of Myelofibrosis is unknown, but researchers believe that genetic mutation causes Myelofibrosis.

  • Age: people above 60 years are more prone to myelofibrosis
  • Environment: Exposure to radiations and petrochemicals increase the risk of developing myelofibrosis
  • Gene mutations: Mutations in JAK2 and MPL genes predisposes you to developing gene mutations.

Myelofibrosis is usually a slowly developing disease. You may be symptom-free for tears. But in the course of cell degeneration the body presents the signs and symptoms such as:

  • Bruising
  • Bleeding
  • Pale skin
  • Anemia, fatigue
  • Fever and bone pain
  • Shortness of breath
  • Increased rate of infections
  • Pain below the ribs mainly on the left side

Other complications caused by myelofibrosis are;

  • Gout
  • Bleeding
  • Severe pain
  • Acute leukemia
  • Pain near joints and bones
  • Abnormal growths in other parts of body

For diagnosing Myelofibrosis, your doctor performs a physical examination to check the enlargement of liver and spleen, a bone marrow examination and a complete blood test.

Complete blood count : People with Myelofibrosis presents with anemia. You may have ‘teardrop-shaped’ red blood cells, higher numbers of white blood cells and platelets. If you are in more advanced stages then your white blood cell counts would be very low.

Bone marrow examination : Bone marrow examination gives the information about the mutations in specific genes such as JAK2. Cytogenic and molecular analysis of blood is performed to confirm the provisional diagnosis.

The main target of the treatment is to increase the quality of life by reducing the symptoms. In case if you are young, you may go for a bone marrow or stem cell transplantation that may cure the disease. Other treatment choice would include:

  • Splenectomy
  • Radiation and chemotherapy
  • Blood transfusions and anti-anemic drugs
  • Medications that target a genetic mutation

When to call a doctor?

You must call your doctor if you have symptoms associated with myelofibrosis or if you notice any abnormal bleeding, shortness of breath, or jaundice symptoms such as yellowing of the white of eyes.

1. How often do I need to visit doctor?

You might need a monthly visit after diagnosing with myelofibrosis in its early stages.

2. What could be my life expectancy?

There is no probable answer. You may live as little as 3 years and it may also be 10 to 15 years or you may live even more by successful bone marrow transplantation.

3. How commonly spleen is affected in myelofibrosis?

85% or more people with myelofibrosis present splenomegaly at the time of diagnosis.

 

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