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Myelofibrosis

Definition


Disease: Myelofibrosis Myelofibrosis
Category: Blood diseases & tumors
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Disease Definition:

The serious bone marrow disorder that disrupts the body's normal production of blood cells is called myelofibrosis. Weakness, severe anemia, fatigue, and often, an enlarged liver and spleen, are caused by the extensive scarring in the bone marrow that results from myelofibrosis.

This condition is also called agnogenic myeloid metaplasia or idiopathic myelofibrosis. Although it mainly develops after the age of 50, myelofibrosis can occur at any age.

Some people with myelofibrosis eventually develop a serious form of leukemia, and many people get progressively worse. However, it is also possible to have myelofibrosis and live symptom-free for years. A variety of options can be involved in the treatment of myelofibrosis, which focuses on relieving symptoms.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Usually, myelofibrosis develops slowly. Many people don't experience signs or symptoms in the very early stages of the condition, however, signs and symptoms may include the following as disruption of normal blood cell production increases:

 

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


Although vague, when someone experiences the following signs and symptoms, he/she should see a doctor:

 

  • Sweating excessively during sleep
  • Having a feeling of unusual fullness in the left abdomen
  • Having other possible signs and symptoms of myelofibrosis
  • Experiencing unexplained weight loss
  • Feeling run-down or tired

Causes:

Hematopoiesis is the name of the process of producing blood cells in the body. With a certain type of cell called a hematopoietic stem cell, this process starts in the bone marrow. The primitive undifferentiated cell that has the ability to replicate and then divide into multiple specialized cells – platelets, white blood cells and red blood cells – is called a stem cell.

There is limited life span for these specialized blood cells. In a continuous, healthy cycle, they die off naturally and are replaced by new cells when they grow old.

HOW DOES MYELOFIBROSIS OCCUR
When the genetic material in a single hematopoietic stem cell changes or mutates, myelofibrosis occurs. An acquired mutation that develops during one's lifetime rather than being present at birth (congenital) is usually the case, but the cause of the mutation is often unclear.

A mutated cell passes along the mutation to the new cells when it replicates and divides. These mutated cells begin to have serious effects on blood production, as more of them are created. An overabundance of white blood cells with varying levels of platelets and a lack of red blood cells is the end result, which causes the anemia characteristic of myelofibrosis.

Myelofibrosis is referred to as a myeloproliferative disease, a type of disease characterized by uncontrolled production of one or more types of blood cells due to the overproduction of white blood cells. A secondary reaction to the activity of the mutated cells is thought to be scarring (fibrosis) within the bone marrow. When the spleen and sometimes the liver trap excess white blood cells circulating through the body, they may become enlarged.

The risk of myelofibrosis may increase due to certain factors, although the cause of this condition is usually not understood:

Exposure to Toxins:
Myelofibrosis has been linked to exposure to carcinogenic chemicals in a few cases such as toluene and benzene, ionizing radiation and thorium dioxide.

Genetic Mutations:
Some people with myelofibrosis have a mutation in the MPL gene or in the JAK2 gene.

Exposure to Contrast Material:
Some people who received an X-ray contrast material called Thorotrast in the 1930s and 1940s have since developed myelofibrosis.

Age:
In people between the ages of 50 and 80, myelofibrosis is diagnosed frequently, though it can affect anyone. With the exception of form that tends to run in families, the condition is usually rare in children.

Complications

Complications:

The following may be the complications that may result from myelofibrosis:

Gout:
The body's production of uric acid, a byproduct of the breakdown of purines which is a substance found naturally in the body and in many foods, is increased by myelofibrosis. Causing joint pain and inflammation (gout), needle-like deposits of the substance in the joints can be caused by overproduction of uric acid. To keep the levels of uric acid normal, one may need medications.

Other organ involvement:
Clumps (tumors) of developing blood cells in other areas of the body may be created by formation of blood cells outside the bone marrow, a condition called extramedullary hematopoiesis. Problems such as bleeding in the gastrointestinal system, coughing or spitting up of blood, compression of the spinal cord, or seizures, may be caused by these tumors. With low-dose radiation, extramedullary blood formation is usually treated.

Pain in the upper left side of shoulder:
Episodes of inflammation or tissue death in the spleen may cause this pain. Symptoms are usually controlled by pain relievers.

Infections:
The overproduction of white blood cells, which help fight infection, can be caused by myelofibrosis. However, these blood cells become ineffective in myelofibrosis, decreasing one's ability to fight off infections, because they aren't fully formed or are mutated.

Acute leukemia:
Acute myelogenous leukemia, a type of blood and bone marrow cancer that progresses rapidly, eventually develops in some people with myelofibrosis.

Increased pressure on blood flowing into the liver:
Through a large blood vessel called the portal vein, blood flow from the spleen enters the liver. High blood pressure in the portal vein (portal hypertension) may be caused by increased blood flow from an enlarged spleen. This in turn can force excess blood into smaller veins in the stomach and esophagus causing them to rupture and bleed. Removal of the spleen may be recommended if problems develop.

Bleeding complications:
Platelet count tends to drop below normal (thrombocytopenia) and platelet function becomes impaired as the disease progresses. Easy bleeding, which is an issue that one should discuss with the doctor if he/she is contemplating any type of surgical procedure, can be caused by an insufficient number of platelets.

Hardening and inflammation of bone tissue:
Leading to severe bone and joint pain and tenderness, myelofibrosis can cause hardening of the bone marrow as well as inflammation of the connective tissue that surrounds the bones.

Treatments:

Treatment usually isn't necessary if one is not experiencing symptoms and doesn't show signs of anemia, an enlarged spleen or other complications. Some people remain symptom-free for years. Because of this, the doctor would probably monitor the health of the patient closely through regular checkups and exams, watching for any signs of disease progression.

Treatment options typically include the following for people with serious symptoms or complications:

Surgical Removal of the Spleen (Splenectomy):
One may benefit from having his/her spleen surgically removed, if he/she doesn't respond to other forms of therapy, or if the size of the spleen becomes painful and begins to cause harmful complications. Excessive bleeding, infection, a higher rate of conversion to acute leukemia, and blood clot formation leading to stroke or pulmonary embolism, are all included in the risks. An abnormal increase in platelet count and liver enlargement may be experienced by some people after the procedure.

Androgen Therapy:
Red blood cell production can be promoted and severe anemia may be improved in some people by taking a synthetic version of the male hormone androgen combined with a corticosteroid medication, such as prednisone. After a month of therapy, people who respond to this treatment may continue on with the androgen and slowly taper off the prednisone. Growth of prostate cancer cells, masculinizing effects in women and liver damage are included in the risks of androgen therapy.

Chemotherapy:
The most commonly used chemotherapy drug in treating myelofibrosis is hydroxyurea. Hydroxyurea can improve night sweats and weight loss, decrease high platelet counts, reduce the size of an enlarged spleen, and possibly reduce bone marrow fibrosis.

Blood Transfusions:
Red blood cell count can be increased and anemia symptoms such as tiredness and weakness can be eased by periodic blood transfusions in case a person has severe anemia. Medications may sometimes help improve anemia so that one doesn't need blood transfusions.

Stem Cell Transplantation:
The only treatment that has the potential to cure myelofibrosis is allogeneic stem cell transplantation, which is the transplantation of stem cell from a suitable donor. However, because it requires high doses of chemotherapy and radiation before transplantation to destroy the diseased cells, it has a high risk of life-threatening side effects. Causing potentially fatal damage (graft-versus-host disease), there is a risk that the new stem cells will react against the body's healthy tissues after the procedure. Other risks include the development of a different cancer later on, cataracts, and organ or blood vessel damage. Because of stability of the disease, age or other health problems, most people with myelofibrosis don't qualify for this treatment.

Thalidomide with Steroids:
Using thalidomide along with prednisone can help improve anemia and white blood cell and platelet counts, reduce spleen size, and improve other systemic symptoms, like shortness of breath, weakness, night sweats and fatigue. This therapy is still fairly experimental, though it can reduce the need for blood transfusions as well.

Radiation Therapy:
Radiation can help reduce the size of the spleen when surgical removal isn't an option, and it may also help a small number of people who have bone pain.

UNDER INVESTIGATION:
A reduced-intensity transplant, also called a nonmyeloablative transplant or mini-transplant, is being studied at present. Relying on the donor's immune system to destroy diseased cells as alternative, lower doses of pre-transplant chemotherapy and radiation are used in this new method. It is hoped that reduced-intensity transplantation will be safer but just as effective as the more aggressive, standard transplant treatments, although it has side effects.

Prognosis:

Not Available

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