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Myelofibrosis – Symptoms, causes, and management options

Myelofibrosis is a rare form of bone marrow cancer affecting the production of blood cells in the body. The condition can cause extensive scarring in the bone marrow, leading to a low platelet count and an increased risk of bleeding. One of the most common symptoms of the disease is anemia. However, the symptoms of myelofibrosis can be managed through treatment. Here are a few key facts about the condition:

Symptoms
Myelofibrosis is a condition that develops gradually. So, in many cases, one may not observe the signs and symptoms in the early stages of the condition. As the disruption in blood cell production increases, one may start to see symptoms. Some of these are:

Shortness of breath

Excessive fatigue

Bruising

Fullness or pain below the ribs, especially on the left side, due to an enlarged spleen

Sudden and easy bleeding

Excessive sweating, especially while asleep

Fever Pain in the bones

If one notices myelofibrosis symptoms, one should immediately consult a doctor to get a treatment plan. Healthcare professionals can offer guidance on diagnostic tests and treatments for relieving the symptoms.

Causes and risk factors
Myelofibrosis develops when the stem cells in the bone marrow develop mutations in their DNA. Stem cells are crucial for the body as they replicate and divide into specialized cells—red blood cells, white blood cells, and platelets. Once the stem cells develop these changes, they will continue to replicate and divide, passing on the mutations to new cells. This can lead to serious effects on blood cell production, reducing the number of red blood cells produced and increasing white blood cell production. While the exact cause for the mutations is not yet known, scientists have been able to highlight a few factors that could lead to the development of myelofibrosis. The risk factors are:

Age: Myelofibrosis is a condition that can affect people of all ages, but studies have found a higher incidence of the condition in older adults over 50.

Radiation exposure: Those who are exposed to high levels of radiation over a long period of time could have an increased risk of developing cancer.

Exposure to industrial chemicals: Studies have also found links between myelofibrosis and increased exposure to certain chemicals like toluene and benzene.

Other blood cell disorders: People with other blood disorders, such as thrombocythemia or polycythemia vera, could also develop myelofibrosis as a secondary complication.

Complications
Here are some of the common complications of the condition:

Increased pressure and flow of blood in the liver: With an enlarged spleen, the pressure and flow of blood leaving the spleen increases. This increase could affect the smaller veins in the stomach and even the esophagus leading to a rupture or bleeding. This can cause complications.

Pain: Myelofibrosis can lead to an enlarged spleen, resulting in the abdomen and the back.

Growths: Myelofibrosis could also lead to the formation of blood cells outside the bone marrow, creating clumps or tumors in other areas of the body. These tumors could lead to other complications, such as bleeding in the gastrointestinal system, spitting blood, compression of the spinal cord, and even seizures.

Bleeding: As the condition progresses, the platelet count can drop. Further, the functioning of platelets can be affected, causing easy bleeding.

Treatment options
While there is no permanent cure for myelofibrosis, the treatment options focus on relieving the symptoms. In some cases, a bone marrow transplant may open up the possibility of curing the disease, but the procedure can often be harsh on the body. This is why a transplant may not be a feasible option for everyone. Healthcare professionals can help one choose the mode of treatment that will benefit the patient. Doctors determine the treatment based on the severity and progression of the disease. They also assign a risk category to determine the aggressiveness of the condition. Those with low-risk conditions may not require immediate treatment, whereas, for high-risk conditions, aggressive treatment may be required. One of the significant symptoms of myelofibrosis is anemia, so doctors can use the following options to treat the symptom:

Blood transfusion: For severe anemia, doctors may recommend blood transfusions to increase the red blood cell count and ease symptoms such as fatigue and weakness.

Androgen therapy: Doctors may also suggest androgen therapy, in which the synthetic version of the male hormone androgen is given to the patient to promote the production of red blood cells and improve anemia symptoms. This treatment option does carry risks like liver damage.

Doctors may also recommend treatment options for enlarged spleens. It is important to discuss the options with one’s doctor to understand the risks and outcomes.

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