Description

Myelofibrosis is a type of cancer of the bone marrow. The bone marrow is the soft matter in the interior of the bone where blood cells are made. Myelofibrosis causes the formation of scar tissue in the bone marrow. This makes it difficult for the bone marrow to produce healthy blood cells.

Myelofibrosis sometimes happens on its own. This is called primary myelofibrosis. Sometimes it is caused by another cell of the blood condition. When this happens, it is called secondary myelofibrosis.

Some people with myelofibrosis have no symptoms and do not need treatment right away. Other people with more-severe forms of the disease may need treatment. The treatment of myelofibrosis usually focuses on relieving the symptoms.

Myelofibrosis belongs to a group of cancers called myeloproliferative neoplasms. These cancers cause the body to produce too many blood cells that don't work well. Myelofibrosis and other myeloproliferative neoplasms may lead to different types of leukemia. Leukemia is a cancer that affects the blood-forming tissues in the body.

Symptoms

Myelofibrosis signs and symptoms may include:

  • Feeling tired, weak, or shortness of breath.
  • Pain or feeling of fullness below the ribs on the left side.
  • Easy bruising.
  • Easy bleeding.
  • Sweating a lot during sleep, so you wake feeling covered in sweat.
  • Fever.
  • Pain in the bones.
  • Feeling full after eating a small amount of food.

When to see a doctor

Make an appointment with a doctor or other health care professional if you have any symptoms that worry you.

Causes

Often it is not clear what are the causes of myelofibrosis. This type of cancer occurs in the bone marrow. The bone marrow is the soft matter in the interior of the bone where blood cells are made.

The bone marrow produces cells called blood stem cells that can become other types of cells. When necessary, these stem cells can become cells of the blood that circulates through the body. Blood stem cells could be:

  • The red blood cells that carry oxygen throughout the body.
  • White blood cells that help fight infections.
  • Platelets help to stop bleeding.

Myelofibrosis is started when the blood stem cells in the bone marrow develop changes in their DNA. A cell's DNA contains the instructions that tell the cell what to do. In healthy blood stem cells, the DNA contains the instructions to activate the cells of the blood in a controlled manner.

In myelofibrosis, the changes in DNA to give different instructions to the stem cells of the blood. The changes, to tell the stem cells from the blood to make more blood cells than the body needs. The blood cells do not function as healthy blood cells.

Myelofibrosis, also causes the formation of scar tissue in the bone marrow. The scarring makes it difficult for the bone marrow to produce healthy blood cells.

The extra blood cells that don't work well and the bone marrow scars can both cause too few healthy blood cells in the body. This leads to the symptoms of myelofibrosis. For example, a feeling of weakness and fatigue that can happen if there are too few red blood cells to transport oxygen in the blood.

Health professionals have discovered some genetic changes in the DNA, which can cause myelofibrosis, including changes in:

  • Janus kinase 2 gene, also called JAK2.
  • The calreticulin gene, also called CALR.
  • Thrombopoietin receptor gene, also called the MPL.

Your health care team can make the test in cancer cells to see if these changes are present. The results can affect your prognosis and treatment options.

Risk factors

Although the cause of myelofibrosis is often not known, the health professionals have found some things that can increase the risk. The risk factors include:

  • The increase of the age. Myelofibrosis can affect anyone, but is most often diagnosed in people over the age of 50 years.
  • Another of the cells of the blood condition. A small portion of people with myelofibrosis develop the condition as a complication of essential thrombocythemia or polycythemia vera.
  • Exposure to benzene. Myelofibrosis has been linked to high levels of exposure to the industrial chemical benzene.
  • The exposure to radiation. People exposed to very high doses of radiation have an increased risk of myelofibrosis.

Complications

The complications that may result from the myelofibrosis include:

Enlargement of the spleen

People with myelofibrosis often have an enlarged spleen. The spleen is hidden below the rib cage on the left side of the belly. This organ has many jobs, including the additional storage of the blood cells. In myelofibrosis, extra blood cells in the bone marrow can accumulate in the spleen. This causes it to swell. The spleen can also start the production of the blood cells if the myelofibrosis causes severe scarring in the bone marrow. This creates more cells that can cause the spleen to swell. When the spleen swells bigger. Very enlarged spleen can cause abdominal pain and feeling of fullness after eating only a small amount of food.

Portal hypertension

Portal hypertension is the medical term for the increase of the pressure in the portal vein. The portal vein carries blood to the liver. If an enlarged spleen is sending more blood than usual to the liver, which can cause pressure in the portal vein. The pressure in the portal vein, can the force of the blood in the smaller veins and cause bleeding in the stomach and the esophagus.

Growths in other areas of the body

If myelofibrosis makes it difficult for the bone marrow to produce blood cells, other organs may begin to make new blood cells. The liver and spleen can start making blood cells. Masses of tissue that can cause the cells to the blood can form in other areas of the body. This can lead to bleeding in the digestive system, cough or coughing up blood, spinal cord compression, or seizures.

Bleeding complications

Myelofibrosis makes it difficult for the body to make healthy platelets. Platelets are the blood cells that help stop bleeding. If you have a few healthy platelets, you may bruise more easily. You may also have an increased risk of dangerous bleeding.

Leukemia

Some people with myelofibrosis develop acute myelogenous leukemia. This is a type of the blood and bone marrow cancer that often gets worse quickly.

Diagnosis

Myelofibrosis diagnosis often begins with a physical exam. Other tests that can help diagnose this cancer of the bone marrow include blood tests and imaging tests. A health care provider may take a sample of bone marrow for testing.

Physical examination

Your health care professional may ask about your symptoms and examine your body. The health care provider may feel your abdomen to check for signs of inflammation in the spleen and the liver.

Blood tests

A health professional may take a blood sample to the test. A blood test used to myelofibrosis is a complete blood count. This test counts the number of cells in a blood sample. In people with myelofibrosis, this test is often low number of red blood cells. White blood cells and the platelet count may be higher or lower than expected.

Imaging tests

Imaging tests make pictures of the inside of the body. For myelofibrosis, a health professional may use the images to see if the spleen and the liver are larger than usual. You can also look for signs that the scar tissue has been replaced with the healthy tissue in the bone marrow. Imaging tests may include computed tomography scans, also called computed tomography, magnetic resonance imaging and ct scans, also called magnetic resonance imaging.

Bone marrow aspiration and biopsy

Bone marrow aspiration and biopsy are procedures that involve the collection of cells of the bone marrow. The cells are sent for analysis.

The bone marrow has a solid part and a liquid part. In a bone marrow aspiration, a needle is used to remove a sample of fluid. In a bone marrow biopsy, a needle is used to collect a small amount of solid tissue and fluid enclosed. The samples are usually taken from the hip bone.

In people with myelofibrosis, this test could find no signs of fibrosis in the bone marrow.

The evidence of the cancer cells, changes in DNA

If cells are found in the blood or the bone marrow, these cells could be tested by changes in the DNA inside cells. The genetic changes are found in the DNA of cancer cells can help your health care team to make a diagnosis. The changes may also help your health care team to make a plan of treatment.

The genetic changes that are the most common in myelofibrosis cells are called JAK2, CALR, and MPL.

Treatment

Treatments for myelofibrosis include blood transfusions and medications, such as chemotherapy and targeted therapy. Other treatments include surgery to remove the spleen, radiation therapy and bone marrow transplant, also called stem cell transplantation.

The goal of treatment for most people with myelofibrosis is to provide relief of the symptoms of the disease. For some, a bone marrow transplant, also called a stem cell transplant, can provide an opportunity for a cure. This treatment can be very hard on the body, and may not be an option for many people.

To find out what myelofibrosis treatments are most likely to benefit you, your health professional may use one or more formulas to assess your condition. These formulas take into account many aspects of your cancer and your general health. A health care provider may assign a risk category, which indicates the severity of your condition.

A lower risk myelofibrosis can grow slowly. The treatment might not be right away, or treatment may focus on the control of symptoms. An increased risk myelofibrosis may be getting worse quickly. The people with the greatest risk myelofibrosis might consider the possibility of treatment more safe, as a bone marrow transplant.

The treatment may not be needed immediately

Myelofibrosis treatment might not be needed right away if you have no symptoms. Instead of giving the treatment, the healthcare professional can closely monitor their health through regular checkups and exams. Some people remain symptom-free for years.

Treatments for anemia

If myelofibrosis is causing severe anemia, your health care professional may recommend treatments, such as:

  • Blood transfusions.
  • Medications that increase the production of blood cells.

Treatments for an enlarged spleen

If an enlarged spleen is causing complications, your health care professional may recommend a treatment. Your options may include:

  • The targeted therapy. Targeted therapy for cancer is a treatment that uses drugs that attack specific chemicals in cancer cells. By blocking these chemicals, specific treatments can cause cancer cells to die. To myelofibrosis, therapy-targeted drugs attack cells with changes in the gene JAK2.
  • Chemotherapy. Chemotherapy treats the cancer with strong medications.
  • The surgery to remove the spleen. The surgery to remove the spleen is called a splenectomy. Can be used if other treatments don't help.
  • The radiation therapy. Radiation therapy treats cancer with powerful rays of energy. Radiation therapy may help reduce the size of the spleen. It can be used if surgery is not an option.

Bone marrow transplantation

A bone marrow transplant, also called a bone marrow stem cell transplantation, involves placing healthy bone marrow stem cells in the body. These cells replace the cells from damage by chemotherapy and other treatments.

To myelofibrosis, the healthy bone marrow stem cells from a donor. A transplant using donor cells is called an allogeneic stem cell transplantation.

Before the transplant, receiving chemotherapy, or radiation treatment to destroy the bone marrow. The healthy stem cells from a donor is placed into your body.

This treatment has the potential to cure myelofibrosis. It also has a high risk of life-threatening side effects. This treatment is not an option for all over the world with myelofibrosis.

Palliative care

Palliative care is a special type of health care that helps people with serious illnesses feel better. If you have cancer, palliative care can help relieve the pain and other symptoms. A team of health professionals providing palliative care. This may include doctors, nurses and other specially trained health professionals. Your goal is to improve the quality of life for you and your family.

Palliative care specialists work with you, your family and your health care team to help you to feel better. They provide an extra layer of support, while you have cancer treatment. You can have palliative care at the same time so strong cancer treatments, such as surgery, chemotherapy or radiation therapy.

When palliative care is used along with all other treatments, people with cancer may feel better and live longer.

Coping and support

Myelofibrosis diagnosis can lead to strong feelings. Some people say that he was surprised, scared, or sad when they came to their diagnosis. With time, each person finds a way of dealing with these feelings. Until you find what works for you, here are some ideas to help you cope with the situation.

Learn enough about your condition to feel comfortable in the decision making

Learn everything you need to know about myelofibrosis. Ask your health care team to recommend some good sources of information to help get you started. As you learn more about your condition, you may feel more confident about making decisions about your care.

Find a good listener

Find someone who is willing to listen to you talk about your hopes and fears can be useful when managing a cancer diagnosis. This could be a friend or family member. A counselor, medical social worker, or a member of the clergy can also provide useful guidance and care.

Consider joining a support group, either in your community or on the internet. A support group for people with cancer can be a source of useful information, practical tips and encouragement.

Take time for yourself

If you have myelofibrosis, you may have a lot of medical appointments. Some days, you may feel sick, even if you don't look sick. And some days, you may be sick of cancer.

Try to set aside time each day for something you enjoy. Find ways to relax. Some people have hobbies that will help take your mind off of cancer for a little while.

Preparing for your appointment

Make an appointment with a doctor or other health care professional if you have any symptoms that worry you. If your healthcare provider thinks that you have myelofibrosis, that person can refer you to a specialist. Often, this is a doctor who specializes in diseases of the blood, called a hematologist.

Appointments can be brief, and being prepared can help. Here is some information that can help you prepare.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test. Make a list of:

  • Its symptoms, including those that do not seem to be related to the reason for your appointment.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your doctor.

Have a friend or family member, if possible, to help you remember the information they give you.

To myelofibrosis, some basic questions to ask your doctor include:

  • Do I have cancer?
  • I need more tests?
  • What are my treatment options?
  • What are the potential risks of these treatment options?
  • Do any of the treatments to cure my cancer?
  • I have a copy of my pathology report?
  • How much time can I take to consider my options for treatment?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?
  • What would happen if I chose not to have treatment?

Do not hesitate to ask other questions.

What to expect from your doctor

Your healthcare provider is likely to ask several questions, such as:

  • When did your symptoms begin?
  • The symptoms been continuous or occasional?
  • How bad are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Myelofibrosis