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Idiopathic Myelofibrosis

Idiopathic Myelofibrosis (MF), also called Agnogenic Myeloid Metaplasia (AMM), is a disorder that causes the gradual replacement of the bone marrow with fibrous (scar) tissue. This leads to a reduction in the number of blood cells produced, and causes the cells that are produced to be dysfunctional. The body recognizes this, and it tries to compensate for it by signaling the spleen and liver to create new blood cells. However, the cells produced by this process do not function properly, and the patient is left with a blood cell deficiency.

Sometimes, patients with MF do not exhibit any symptoms at all. Many patients, however, will present with an enlarged spleen or liver. Patients also may experience symptoms of anemia, including weakness and fatigue. A low platelet count due to MF can result in bleeding and clotting difficulties, and a low number of white blood cells can cause an increased risk of infection. Occasionally these blood cells are high rather than low.

Every year, there are two new cases of MF per 100,000 people. It usually occurs between the ages of 50 and 70 years old, although it can occur at all ages, and it affects both males and females equally. There is no known cure.

Diagnostic Tests

To diagnose MF, your doctor will order a blood test to determine if your bone marrow is producing the correct amount of each type of cell. If the results are abnormal, you may be referred to a hematologist, a doctor who specializes in diagnosing and treating bone marrow and blood disorders. The hematologist may then test you further by doing a bone marrow biopsy. This consists of removing a small amount of bone marrow and examining it for abnormalities.

Treatment Overview

The primary goal of treatment is to control the swelling of the spleen and to provide the body with enough red blood cells. The treatment you receive will depend on your age, health, if you are having any symptoms, and the results of your blood test and biopsy. It may include:

Monitoring

In some patients, the best treatment is to monitor the disorder for any increase in symptoms and have a doctor do periodic blood counts. This may be the only treatment ever needed.

Blood Transfusions

To treat symptoms of anemia, you may be given blood transfusions. Your doctor and nurse will discuss risks associated with blood transfusions with you.

Hormonal Therapy

Hormonal therapy is the use of medication to increase the number of red blood cells in the body. Androgens, including oxymetholone and fluoxymesterone, are one type of medication your doctor might prescribe. Glucocorticoids, another type of hormone, are sometimes given in conjunction with the androgens to increase the length of time red blood cells will live.

Chemotherapy

Chemotherapy treatment uses medications to slow the production of blood cells in the body. One type of chemotherapy used to treat MF is hydroxyurea.

Surgery

Surgery is used to remove the spleen if it becomes so enlarged that it causes breathing difficulty or heart problems. This operation is called a splenectomy.

External Beam Radiation Therapy

If the spleen becomes enlarged, your doctor may recommend External Beam Radiation Therapy. This treatment uses high energy x-rays to kill cells.

Biological Therapy

Biological therapy, or immunotherapy, uses natural substances to bolster your own immune response to the disorder. Alpha-interferon, for example, is a medication used to reduce the white blood cell and platelet counts if they are too high.

Other Medications

Other medications might be used to control symptoms of MF. For example, some doctors may prescribe medications such as allopurinol to prevent a high uric acid level, a condition that causes gout.

Managing Idiopathic Myelofibrosis

In addition to the treatment recommended by your doctor, there are steps you can take to help reduce or prevent the symptoms of MF:

Avoid exposure to crowds or to people with colds or contagious illnesses if your leukocytes are low.
Practice good hygiene, including frequent hand washing.
Brush your teeth regularly, bathe daily, and pay special attention to hard-to-clean areas such as skin folds of the rectal area.
Avoid bruising or bumping yourself.
Use an electric razor when shaving and be cautious when using nail trimmers, knives, etc.
Wear hard-soled shoes, gloves, and long pants when working outside (i.e. gardening).
Use a sponge toothbrush if you have problems with gum bleeding. Your doctor or nurse can tell you if you need to use one and where it can be acquired.
Avoid aspirin or aspirin-like medications (for example, Motrin, ibuprofen, or other anti-inflammatory drugs) unless your doctor has told you otherwise. These medications can affect blood clotting. Also, be sure to inform your doctor of all over-the-counter medications that you take (including vitamins, herbs and dietary supplements).
Concentrate on having a well-balanced diet during this time. This helps your body to make new red blood cells.
Sleep and rest between activities to conserve energy.
Engage in light exercise, such as walking, to stimulate circulation and improve energy levels.
Inform your dentist and all other medical personnel that you have this disorder. There may be increased risk of infection and bleeding during some procedures.

It is important that you be alert for any change or increase in symptoms. If this occurs, it may mean that you need additional or different treatments, and you should contact your doctor right away. The following symptoms require prompt attention:

Enlargement of Spleen or Liver Symptoms

abdominal swelling that you see
abdominal fullness or pain that you feel
weight loss

Anemia Symptoms

weakness
shortness of breath
fatigue
pale appearance
rapid heart rate

Infection Symptoms

fever exceeding 100.4° F (38.0 C)
chills
night sweats
cough
sore throat
rectal soreness
mouth or lip sores
pain during urination
sores that do not heal, have drainage, or are swollen, red, and warm to the touch
stiff neck

Bleeding Symptoms

easy bruising
unusually heavy or prolonged bleeding
bleeding for no apparent reason
severe headache or visual changes
stiff neck
joint pain
petechiae, or tiny areas of pinpoint bleeding on the skin of the arms or legs

Follow-Up Care and Prognosis

MF requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment.

Idiopathic myelofibrosis is a disorder that cannot be cured, but, with treatment, the disorder can be controlled. Some patients may only need minimal supportive care and observation, while other patients may require more intensive treatment. In a very small percent of patients, MF may evolve into other chronic disorders or transform into an acute leukemia.

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