What is Myelofibrosis?
This is a bone marrow disorder that is very serious and that disturbs the regular creation of blood cells. This will cause scarring in the bone marrow that is very extensive and leads to anemia that is severe, fatigue, and weakness as well as in some cases an enlarged liver and spleen.
Myelofibrosis is actually a type of chronic leukemia – cancer that upsets the tissues that are blood-forming in the body. This disorder can happen on its own (primary myelofibrosis) or it may occur because of other disorders of the bone marrow (secondary myelofibrosis).
Numerous individuals with myelofibrosis progressively get worse and certain ones can eventually develop a much more serious form of leukemia. However, it is possible as well to have myelofibrosis and live a symptom-free life for many years. The treatment for this condition that focuses on symptoms relief may comprise an assortment of options.
Myelofibrosis Symptoms
Myelofibrosis normally develops quite slowly. In the very first stages, most individuals do not experience symptoms or signs. However, as the interruption of standard production for blood cells intensifies, symptoms and signs can consist of:
- Feeling weak, tired or short of breath, normally because of anemia
- Enlarged liver
- Fullness or pain below the ribs on the left side, due to enlarged spleen
- Pale skin
- Easy bleeding
- Easy bruising
- Fever
- Night sweats
- Frequent infections
- Bone pain
An individual should make a visit to see their doctor when the symptoms and signs are tenacious enough to begin to worry you.
Myelofibrosis Causes
Myelofibrosis develops when a mutation that is genetic develops in the stem cells for blood. These stem cells are able to copy as well as split into the several precise cells making up the blood – red cells, white cells as well as platelets.
It really is not known exactly the cause of this hereditary alteration in the stem cells of blood. As the stem cells that are mutated copy and split, they are passing along this mutation to the fresh cells. As this copy and splitting occurs time after time, more mutated cells are produced and begin to have effects which are serious on the production of blood. The endgame is normally a shortage of red blood cells – that causes the characteristic anemia associated with myelofibrosis, as well as an excess of white cells with various levels of platelets. For individuals with myelofibrosis, the bone marrow that is normally spongy will become scarred.
The mutation of the gene that happens in the majority of individuals with myelofibrosis is often denoted as JAK2. Additional gene mutations can also be linked with myelofibrosis.
While the reason for myelofibrosis is not often known, there are some factors that are understood that can intensify your risk and they consist of:
Age
Myelofibrosis may touch anyone however it is more often found in individuals in the middle of the ages of fifty and seventy.
Additional problem with blood cells
A slight number of individuals who have myelofibrosis developed the disorder as a problem developed from having thrombocythemia or polycythemia vera.
Contact with definite chemicals
Myelofibrosis has been associated with contact to some industrial chemicals for instance benzene and toluene
Contact with radiation
Individuals with exposure to high rates of radiation, for instance survivors of nuclear bomb blasts have shown an increased likelihood of developing myelofibrosis. Many individuals who had contact with radioactive contrast matter known as Thorotrast, used from the 20s to the 50s, has in the meantime developed this condition.
Myelofibrosis Complications
Myelofibrosis can lead to the following complications:
Pressure increase on blood flow into the liver
Typically the blood flowing from the spleen enters the liver thru a big blood vessel known as the portal vein. Any amplified flow of blood from a spleen that is enlarged may lead to blood pressure that is too high in this portal vein which is known as portal hypertension. This force can then force excessive blood into veins that are smaller in the esophagus as well as the stomach, possibly causing the rupture and bleeding of the veins.
Pain
A spleen that is enlarged severely often creates pain in the back and abdomen.
Tumors in other regions of the body
The creation of blood cells separate from the bone marrow is known as extrameduallary hematopoiesis can develop clumps or tumors of blood cells developing in other parts of the body. Such tumors can create problems for instance bleeding in the gastrointestinal system, spitting or coughing of blood, compression of the spinal column or seizures.
Bleeding difficulties
As this disorder progresses, the platelet count is incline to drop below the normal level – this is known as thrombocytopenia and the platelet function also becomes diminished. Numbers of platelets that are insufficient often leads to bleeding easily which is an issue that the individual and the doctor will need to discuss prior to any sort of surgical technique being performed.
Infections
Myelofibrosis also can increase the formation of too many white blood cells that help fight infection. But with this condition, these cells often are not totally formed or they are mutated so that these cells are ineffective, and decreases the ability to fight infections.
Inflammation and hardening of bone matter
Myelofibrosis can cause bone marrow to harden and also inflammation of any connective tissue surrounding the bones, causing tenderness and severe joint and bone pain.
Gout
This condition intensifies the body’s manufacturing of uric acid which is a result of the breaking down of purines – a naturally found substance in the body and in some foods. The excessive production of uric acid leads to deposits that are needle-like in the joints, causing pain and inflammation to these joints. This is known as gout.
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Acute leukemia
Many people with the diagnosis of myelofibrosis ultimately develop “acute myelogenous leukemia” that is a form of blood/bone marrow cancer that advances swiftly.
Myelofibrosis Treatment
In those individuals who don’t have symptoms, routine checkups will normally reveal a spleen that is enlarged as well as blood tests that are abnormal can raise fears of some type of medical difficulty. If you go to your physician because of any symptoms, a physical as well as blood tests are normally the first actions the physician will take.
To make a confirmation of the myelofibrosis diagnosis, the individual can have image studies of the bones, spleen as well as the liver and examination a bone marrow sample.
Physical examination
The physician will complete a detailed physical exam. This should include a checkup of vital signs such as blood pressure and pulse, as well as a check of the lymph nodes, abdomen and spleen.
Tests of blood
With myelofibrosis, a total blood count normally shows an abnormally low level of red cells, an indication of anemia. Platelet and white cell counts are frequently abnormal also. Usually, white cell levels are higher than usual, while with some individuals these counts can be standard or in some cases lower than standard. The same can be true of platelet counts.
Imaging exams
Imaging tests for instance ultrasound, can be done to define the size of the spleen as well as others, such as MRI, may identify any changes in the bones that will indicate myelofibrosis.
Bone marrow exam
Biopsy of bone marrow as well as aspiration may confirm the myelofibrosis diagnosis. With this biopsy, a sample of the hardened bone marrow from the hip is drawn with a needle. During this procedure, another needle type is used to get a sample of the portion of the bone marrow that is liquid. These samples are reviewed in the lab to define the number of as well as the types of cells produced. Also by studying the components of the cells of the bone marrow that are genetic can reveal cells in the mutations that can help to exclude any other types of disorders of the bone marrow.
Tests for genes
With a sample of bone marrow a blood analysis can be done in the lab to look for any mutations of the genes in the blood cells that are normally associated with myelofibrosis.
If you are not having any symptoms and do not show any signs of anemia, enlarged spleen or other problems, treatment is normally not necessary. In its place, the physician might want to just monitor closely your health thru checkups and exams that are regular and that watch for any signs of progression of the disease. Many individuals remain free of symptoms for years.
If this disease is causing anemia that is severe, the individual can consider several treatments, for instance:
Blood transfusions
If the anemia is severe, the individual can have periodic transfusions of blood that may raise the red cell count to ease the symptoms of anemia, such as weakness and fatigue. Often drugs can help to control anemia so that blood transfusions are not needed.
Androgen therapy
This is a synthetic form of the male hormone known as androgen that can promote red cell production as well as can control severe anemia in some individuals. Androgen therapy has risk, for instance damage to the liver, masculinizing effects in women as well as growth of prostate cancer in males.
Thalidomide as well as related drugs
Thalidomide and the associated drug lenalidomide can help to better the counts of blood cells as well as can also relieve the spleen that is enlarged. These medications can be used with steroid medications. Thalidomide as well as related medications carries a risk of birth defects which are serious and may need exceptional defenses. This form of therapy is being studied further in clinical trials.
Treatments for enlarged spleen
If there is a spleen that is enlarged that is creating complications, the physician can recommend several treatments. The options can include:
Splenectomy –surgical removal of the spleen
If the spleen size is at the point of being painful as well as beginning to create detrimental complications and if no form of therapy is working, the individual can usually benefit from having the spleen removed surgically. Risks to this surgery can include excessive bleeding, infection as well as blood clot creation that can lead to pulmonary or stroke embolism. Normally after this procedure, many individuals experience enlargement of the liver as well as an abnormal raise in count of platelet.
Chemotherapy
Drugs for chemotherapy can reduce the spleen size if enlarged and can relieve many related symptoms, for instance pain.
Radiation therapy
Radiation using beams that are high-powered, for instance x-rays to eradicate cells. Radiation therapy also may help reduce the spleen size, especially if surgical elimination is not a possibility.
Allogeneic stem cell transplantation
A stem cell transplant from a donor who is suitable is the lone therapy that has the probability to cure myelofibrosis. But it can also have a extraordinary risk of life-threatening side effects. Most individuals with myelofibrosis, because of stability of disease, age or other problems with health, are not eligible for this treatment.
Myelofibrosis Prognosis
Myelofibrosis is a disorder that is progressive, and in most cases is irreversible. The prognosis rest on the individual’s age, red and white count, and bone marrow cytogenetic results. The best prognosis is living at least 15 years with the poor prognosis being 12 to 18 months.
