Myelofibrosis – What it is, Symptoms and Treatments of this condition. Also, myelofibrosis is a serious bone marrow disorder that disrupts your body’s normal production of blood cells. The result is extensive scarring in your bone marrow, leading to severe anemia , weakness, fatigue, and often an enlarged spleen.
Myelofibrosis is an uncommon type of chronic leukemia – a cancer that affects the blood-forming tissues in the body. Myelofibrosis belongs to a group of diseases called myeloproliferative disorders. Many people with myelofibrosis get progressively worse, and some eventually develop a more severe form of leukemia.
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However, it is also possible to have myelofibrosis and live symptom-free for years. Treatment for myelofibrosis , which focuses on relieving symptoms, can involve a variety of options. So, check now Myelofibrosis – What it is, Symptoms and Treatments:
What is Myelofibrosis: Myelofibrosis (MF) is a rare condition that affects the bone marrow. Bone marrow is where our blood cells are made. In MF, scar tissue builds up within the bone marrow and blood cells are not made properly. Other parts of the body, usually the spleen and liver, start making blood cells.
Causes of Myelofibrosis: Myelofibrosis occurswhen blood stem cells develop a genetic mutation. Blood stem cells have the ability to replicate and divide into the multiple specialized cells that make up blood – red blood cells, white blood cells and platelets. It is unclear what causes the genetic mutation in blood stem cells.
As the mutated blood stem cells replicate and divide, they are mutated into the new cells. As more and more of these mutated cells are created, they begin to have serious effects on blood production. The end result is usually a lack of red blood cells – which causes the characteristic anemia of myelofibrosis – and an overabundance of white blood cells with varying levels of platelets.
In people with myelofibrosis , the normally spongy bone marrow becomes scarred. Several specific gene mutations have been identified in people with myelofibrosis . The most common is the Janus kinase 2 (JAK2) gene. Knowing whether the JAK2 gene or others are associated with your myelofibrosis helps determine your prognosis and treatment.
Symptoms of Myelofibrosis: Myelofibrosis usuallydevelops slowly. In its early stages, many people experience no signs or symptoms. As the disruption of normal blood cell production increases, signs and symptoms of myelofibrosis may include:
- Feeling tired, weak, or short of breath, usually because of anemia
- Pain or fullness below the ribs on the left side, due to an enlarged spleen
- easy bleeds
- easy greeting
- Excessive sweating during sleep (night sweats)
- bone pain
Make an appointment with your doctor if you have any persistent signs and symptoms that worry you.
Myelofibrosis Risk Factors: While the cause of myelofibrosis is often unknown, certain factors are known to increase your risk:
- Advanced age. Myelofibrosis can affect anyone , but it is most often diagnosed in people over age 50.
- Another blood cell disorder. A small portion of people with myelofibrosis develop the condition as a complication of essential thrombocytosis or polycythemia vera.
- Exposure to certain chemicals. Myelofibrosis has been linked to exposure to industrial chemicals such as toluene and benzene.
- Radiation exposure. People exposed to high levels of radiation, such as atomic bomb survivors, are at increased risk of myelofibrosis . Some people who were given a radioactive contrast material called Thorotrast, used until the 1950s, developed myelofibrosis .
Complications of Myelofibrosis: Complications that can result from Myelofibrosis include:
- Increased pressure on blood flow to your liver. Normally, blood flow from the spleen enters the liver through a large blood vessel called the portal vein. Increased blood flow from an enlarged spleen can lead to high blood pressure in the portal vein (portal hypertension). This, in turn, can force excess blood into smaller veins in the stomach and esophagus, potentially causing these veins to rupture and bleed.
- Ache. A severely enlarged spleen can cause abdominal pain and back pain.
- Growths in other areas of your body. The formation of blood cells outside the bone marrow (extramedullary hematopoiesis) can create clusters (tumors) of blood cells developing in other areas of your body. These tumors can cause problems such as bleeding in the gastrointestinal system, coughing or spitting up blood, spinal cord compression, or seizures.
- Bleeding complications. As the disease progresses, your platelet count tends to drop below normal (thrombocytopenia) and platelet function is impaired. An insufficient number of platelets can lead to easy bleeding – an issue that you and your doctor will want to discuss if you are contemplating any type of surgical procedure.
- Painful bones and joints. Myelofibrosis can lead to hardening of the bone marrow and inflammation of the connective tissue that is found around the bones. This can cause nasal and joint pain.
- Acute leukemia. Some people with myelofibrosis eventually develop acute myeloid leukemia , a type of blood and bone marrow cancer that progresses quickly.
Diagnosing Myelofibrosis: Tests and procedures used to diagnose Myelofibrosis include:
- Physical exam. Your doctor will perform a physical exam. This includes a check of vital signs such as pulse and blood pressure, as well as checks of your lymph nodes, spleen, and abdomen.
- Bloodtests. In myelofibrosis , a complete blood count usually shows abnormally low levels of red blood cells, a sign of anemia common in people with myelofibrosis . White blood cell and platelet counts are also abnormal. White blood cell levels are often higher than normal, although in some people they may be normal or even lower than normal. The platelet count may be higher or lower than normal.
- Image tests. Imaging tests such as X-rays and magnetic resonance imaging (MRI) may be used to gather more information about your myelofibrosis .
- Bone marrow exam. Bone marrow biopsy and aspiration can confirm the diagnosis of myelofibrosis . In a bone marrow biopsy, a needle is used to draw a sample of hardened bone marrow from your hipbone. During the same procedure, another type of needle may be used to take a sample of the liquid portion of the bone marrow. The samples are studied in a laboratory to determine the numbers and types of cells found.
- Gene tests. A sample of your blood or bone marrow can be analyzed in a laboratory to look for genetic mutations in blood cells associated with myelofibrosis .
Myelofibrosis Treatments: If you are not experiencing symptoms and do not show signs of anemia , an enlarged spleen, or other complications, treatment is usually not necessary. Instead, your doctor will likely monitor your health closely through regular checkups and checkups, watching for any signs of disease progression. Some people remain symptom-free for years.
Treatments That Target Gene Mutations: Researchers are working to develop drugs that target the JAK2 gene mutation thought to be responsible for myelofibrosis . The first of these drugs approved by the Food and Drug Administration is ruxolitinib (Jakafi). Ruxolitinib and other drugs developed and tested in clinical trials have been helpful in shrinking an enlarged spleen and reducing symptoms associated with myelofibrosis .
It is not yet clear whether these drugs can help people with myelofibrosis live longer. But preliminary indications from clinical trials are promising. Ruxolitinib works by stopping the action of all JAK-related genes in the body, including those found in healthy and diseased cells. As healthy cells are affected, side effects such as bleeding, infection, bruising, dizziness and headaches can occur.
Anemia Treatments: If myelofibrosis is causing severe anemia , you may want to consider treatment such as:
- Blood transfusions. If you have severe anemia , periodic blood transfusions can increase your red blood cell count and relieve anemia symptoms such as fatigue and weakness. Medications can sometimes help improve anemia .
- Androgen therapy. Taking a synthetic version of the male hormone, androgens can promote red blood cell production and can improve severe anemia in some people. Androgen therapy has risks, including liver damage and masculinizing effects in women.
- Thalidomide and related drugs. Thalidomide (Thalomid) and the related drugs lenalidomide (Revlimid) and pomalidomide (Pomalyst) can help improve blood cell counts and can also relieve an enlarged spleen. These medications can be combined with steroid medications. Thalidomide and related drugs pose a risk of serious birth defects and require special precautions. This type of treatment is being studied in clinical trials.
Enlarged Spleen Treatments: If an enlarged spleen is causing complications, your doctor may recommend treatment. Your options may include:
- Chemotherapy. Chemotherapy drugs can reduce the size of an enlarged spleen and relieve related symptoms, such as pain.
- Radiation therapy. Radiation uses high-powered beams, like X-rays, to kill cells. Radiation therapy can help reduce the size of the spleen when surgical removal is not an option.
- Surgical removal of the spleen (splenectomy). If the size of your spleen becomes so large that it causes pain and begins to cause harmful complications — and if you don’t respond to other forms of therapy — you may benefit from having your spleen surgically removed. Risks include infection, excessive bleeding, and blood clots that lead to stroke or pulmonary embolism. After the procedure, some people experience an enlarged liver and an abnormally high platelet count.
- Targeted drug therapy. Ruxolitinib, which targets the gene mutation found in most cases of myelofibrosis , can be used to reduce symptoms of an enlarged spleen.
Stem Cell Transplantation: Allogeneic stem cell transplantation – the transplantation of stem cells from a suitable donor – is the only treatment that has the potential to cure myelofibrosis . But it also has a high risk of life-threatening side effects. Many people with myelofibrosis , due to age, disease stability, or other health issues, do not qualify for this treatment.
Before a stem cell transplant, also called a bone marrow transplant, you are given very high doses of chemotherapy or radiation to destroy your diseased bone marrow. Then you receive stem cell infusions from a compatible donor.
After the procedure, there is a risk that the new stem cells will react against healthy tissues in your body, causing potentially fatal damage (graft-versus-host disease). Other risks include damage to organs or blood vessels, cataracts, and developing a different cancer later on.
Doctors are studying a reduced-intensity transplant, also called a non-myeloablative transplant or mini-transplant. Reduced-intensity transplants use lower doses of pre-transplant chemotherapy and radiation. While reduced-intensity transplantation has side effects, doctors hope it will one day be a safer option for older adults.
Supportive (palliative) care: Palliative care is specialized medical care that focuses on providing relief from pain and other symptoms of a serious illness. Palliative care specialists work with you, your family and your other doctors to provide an extra layer of support that complements your ongoing care. Palliative care can be used during other aggressive treatments, such as surgery, chemotherapy, or radiation therapy.
When palliative care is used along with all other appropriate treatments, people with cancer can feel better and live longer. Palliative care is provided by a team of specially trained doctors, nurses and other professionals.
Palliative care teams aim to improve the quality of life for people with cancer and their families. This form of treatment is offered along with curative or other treatments you may receive.
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Prevention of Myelofibrosis: Myelofibrosis cannot be prevented, but it can be diagnosed quickly with periodic examinations and proper medical follow-up. Be sure to talk to a doctor if you experience any unusual symptoms for long periods of time.