Myelofibrosis

Myelofibrosis is a type of bone marrow cancer that affects the body's ability to produce blood cells. This condition causes the growth of scar tissue in the bone marrow, disrupting the production of blood cells .

Patients with myelofibrosis often do not feel any symptoms at the onset of the disease. However, as the disturbance in the production of blood cells in the bone marrow worsens, sufferers will experience symptoms of anemia, such as paleness and fatigue, and bleeding easily.

Causes of Myelofibrosis

Myelofibrosis occurs when stem cells in the bone marrow have mutations or changes to their DNA (genes). These stem cells are supposed to have the ability to divide into the special cells that make up blood, such as red blood cells, white blood cells, and platelets.

After that, the mutated blood stem cells will replicate and divide so that more and more cells will change. This condition causes serious effects on blood cell production and leads to the growth of scar tissue in the bone marrow.

Although often associated with mutations or changes in genes, myelofibrosis is not inherited from parents.

There are several risk factors that increase the risk of this gene mutation, namely:

• Increasing age
  Myelofibrosis can affect anyone, but most often occurs in people over 50 years of age.
• Suffering from blood cell disorders
  People with blood cell disorders, such as essential thrombocythemia or polycythemia vera , can suffer from myelofibrosis.
• Exposure to certain chemicals
  The risk of developing myelofibrosis will increase if you are often exposed to industrial chemicals, such as toluene and benzene.
• Exposure to radiation
  People who are exposed to radiation at very high levels are more at risk of developing myelofibrosis.

Symptoms of Myelofibrosis

Symptoms of myelofibrosis are often not visible at the start, so many sufferers are not aware of the appearance of this disease. However, there are some symptoms that appear as the disease progresses and begins to interfere with blood cell production. These symptoms include:

• Symptoms of anemia , such as fatigue, pale skin, to shortness of breath
• Pain in the area around the ribs, because the spleen is enlarged
• Fever
• Sweat often
• No appetite
• Weight loss
• Skin bruises easily
• Nosebleed
• Bleeding gums

When to see a doctor

Sometimes sufferers do not realize that the complaints they experience are symptoms of myelofibrosis. Therefore, check with your doctor if you experience the above symptoms, especially if they don't get better.

Patients with myelofibrosis need to carry out routine control to the doctor to monitor the progress of the disease. Routine control also aims to anticipate and detect early complications.

Diagnosis of Myelofibrosis

The doctor will begin the examination by asking about the patient's symptoms, then examine the pulse, blood pressure, as well as the abdomen and lymph nodes.

The physical examination aims to look for signs of myelofibrosis, such as pale skin due to anemia or swelling of the spleen organ. In addition, the doctor will also carry out the following supporting examinations:

• Blood test The
  doctor will do a complete blood count test to find out the number of red blood cells, white blood cells, and platelets. The suspicion of myelofibrosis will be even stronger if the number of blood cells is too much or too little, and abnormally shaped blood cells are found.
• Scanning
  An ultrasound scan of the abdomen aims to detect an enlarged spleen. Enlargement of the spleen can be a sign of myelofibrosis.
• Bone marrow aspiration and biopsy Bone marrow
  biopsy and aspiration is performed by taking a sample of the patient's blood and bone marrow tissue using a fine needle. The tissue sample will then be examined in the laboratory to see if there is a problem.
• Genetic
  tests Genetic tests are carried out by taking a sample of the patient's blood or bone marrow to be examined in the laboratory. This examination aims to look for gene changes in blood cells associated with myelofibrosis.

Treatment of Myelofibrosis

After the patient is confirmed to have myelofibrosis, the doctor will discuss with the patient and his family about the treatment steps that need to be taken. The following are available treatment methods to treat myelofibrosis:

• Blood transfusions Regular blood
  transfusions can increase the number of red blood cells and relieve symptoms of anemia.
• Medications
  Medications such as thalidomide and lenalidomide can help increase blood cell counts and shrink the spleen. These drugs can be combined with corticosteroid drugs .
• JAK2 inhibitor drugs JAK2 inhibitor
  drugs are given to slow or stop gene mutations that cause cancer cell growth.
• Chemotherapy
  Chemotherapy is the administration of drugs to kill cancer cells. This drug can be given in tablet form or by injection.
• Radiotherapy
  Radiotherapy is the use of special beam radiation to kill cells. Radiotherapy aims to help reduce the size of the spleen.
• Bone marrow transplant
  Bone marrow transplant is done if myelofibrosis is very severe. In this procedure, the doctor will replace damaged bone marrow with healthy bone marrow.

Myelofibrosis complications

Myelofibrosis can cause serious complications if not treated immediately. Some of these complications are:

• Increased blood pressure in the veins of the liver (portal hypertension)
• Chronic back pain due to enlarged spleen
• Tumor growth in certain parts of the body
• Bleeding in the digestive tract
• Myelofibrosis changes to acute leukemia

Prevention of Myelofibrosis

Myelofibrosis cannot be prevented, but the risk can be reduced by undergoing regular health checks. That way, myelofibrosis can be detected earlier and can be treated immediately.

Exposure to chemical compounds or radiation in the work environment is also at risk of causing myelofibrosis. If you work in a place that is frequently exposed to chemicals or radiation, use personal protective equipment that complies with work safety standards and carry out regular employee medical check - ups .