Myelofibrosis

Myelofibrosis: Causes, Symptoms & Treatment

Myelofibrosis is a rare kind of bone marrow tumor that hinders the body’s ability make healthful blood cells. This disease causes scarring (fibrosis) within the bone marrow and results to severe anemia a low platelet count and an increased size of the lymph nodes.

In the category of blood cancers referred to as myeloproliferative Neoplasms myelofibrosis could be a separate entity (primary) or arise from other conditions such as essential thrombocythemia and polycythemia vera. While some individuals are symptom free for decades, others require urgent and severe treatment.

Symptoms of Myelofibrosis

In the beginning there are many patients who exhibit no obvious signs. As the disease develops, symptoms could include:

• A persistent tiredness or weakness (due in part to anemia)

• Breathing shortness

• A feeling of pain or swelling underneath the left ribs (from an over-extended the spleen)

• The possibility of bleeding or bruising is very easy.

• Night sweats

• Fever

• Bone pain

What Causes Myelofibrosis?

Myelofibrosis can be caused by the genetic changes that affect the stem cells of bone marrow, primarily it is caused by the JAK2 modification. The abnormal cells multiply in uncontrolled ways cause blood loss, cause disruption to normal blood production and can cause fibrosis in the bone marrow.

Although the exact cause for the mutations is unclear however, several potential risk factors are recognized.

Risk Factors

You are more likely to be diagnosed with myelofibrosis if:

• Are you over the age of 50?

• Are you a victim of other blood disorders, such as the essential blood clots as well as polycythemia vera

• Have you been exposure to chemical compounds used for industrial use, such as the benzene and toluene

• Have you been exposed to radiation at high levels?

Potential Complications

If untreated or poorly managed, myelofibrosis could result in:

• Portal Hypertension A vein with high blood pressure supplies blood into the liver as a result of the increased flow of blood from the spleen.

• Spleen swelling: Pain in the back or abdomen because of pressure on the spleen.

• Extramedullary hemotopoiesis The production of blood cells outside of the marrow, which leads to tumor-like growths within organs, spinal cord, or the lungs.

• Bleeding Issues Because of lower platelet count (thrombocytopenia).

• Advancement to Acute Myeloid Leukemia (AML): A more aggressive type of blood cancer which can result from myelofibrosis that is advanced.

Diagnosis and Treatment

Diagnosis is based on:

• Tests of blood to measure the levels of white, red, and levels of platelets

• Bone Marrow biopsy to determine the structure of the marrow and fibrosis

• Tests for gene expression to determine if there are mutations such as JAK2, CALR or MPL

Treatment Options

There is no current treatment for myelofibrosis. the treatment focuses on controlling symptoms and enhancing quality of life. Depending on the severity the treatment options consist of:

• Treatments such as JAK inhibitors (e.g., Ruxolitinib) to shrink the size of the spleen and alleviate symptoms

• Transfusions of blood to treat severe anemia

• Chemotherapy in high-risk situations

• Stem cells transplant (for young patients suffering from an aggressive disease)

The Myelofibrosis Patient

Monitoring on a regular basis and creating a customized treatment plan can help control symptoms and avoid complications. Regular check-ups with a Hematologist are essential.

Conclusion

Myelofibrosis can be a rare but serious illness that requires prompt diagnosis and proper treatment. Thanks to advances in treatment, a lot of people have a productive life while managing the disease. If you’re experiencing constant bleeding, fatigue that is not explained or an overly large stomach, see a healthcare doctor for a thorough examination.