In myelofibrosis, the problem occurs in the functioning of stem cells of bone marrow. The reason for causing this problem remains unknown; some factors increase the risk of developing myelofibrosis.

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Who Is At Risk For Myelofibrosis?

Although the exact cause of the disease is not known, however, research indicates that various factors increase the risk of developing myelofibrosis. These factors include-

Age- Although the disease is found in the individuals of all the age group, in most cases, the disease is found in the people who are above 50 years.

Exposure To Toxic Chemicals- People with chronic exposure to chemicals especially the organic chemicals such as benzene are at increased risk of developing this disorder. This is most commonly seen in those people who work in chemical industries.

Radiation Exposure- Myelofibrosis is caused due to the mutation in the stem cells leading to abnormal growth. Exposure to radiation is the most common cause of cell mutation.

Presence Of Other Blood Disorder- Primary myelofibrosis occurs when the stem cells get abnormal. However, the risk of development of secondary myelofibrosis increases with the presence of other blood disorders such as polycythemia vera and essential thrombocythemia. People with polycythemia vera are at increased risk of developing myelofibrosis as compared to people with essential thrombocythemia. (1)

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Smoking- Cigarette smoking is one of the major contributing factors for developing Myeloproliferative disorders including myelofibrosis, essential thrombocythemia, and polycythemia vera. According to a study, smoking cigarette triples the risk of developing these conditions.

Chemotherapy- Chemotherapy for treating malignant condition affects the bone marrow cells and may increase the risk of myelofibrosis.

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What Are The Complications Of Myelofibrosis?

Following are the complication of myelofibrosis-

Lumps- Lumps are formed in other areas of the body due to the extramedullary formation of blood cells. These lumps or tumors may result in bleeding in areas of the body such as GIT.

Increase Pressure On Liver Vessels- The blood from the spleen to liver flows at a higher rate. This may cause rupture of the small vessels present in the liver.

Acute Leukemia- This is the life-threatening complication of myelofibrosis. Myelofibrosis in many patients progresses to cause Acute Myeloid Leukemia. (1)

Symptoms Of Myelofibrosis

Many people suffering from myelofibrosis does not have any symptoms and the disease is diagnosed when the blood testing is done for some disease. However, people, in whom the disease progresses at a rapid rate, experience various symptoms that may affect the quality of their life. Following are the symptoms experienced by the patient of myelofibrosis patients-

Anemia- Myelofibrosis is the condition in which there is reduced the production of RBC. Thus, the level of hemoglobin in these people is low and they develop anemia during the progression of the disease. The patient experiences fatigue and weakness due to low supply of oxygen.

Recurring Infection- Bone marrow stem cells are also responsible for producing WBC. These cells are part of the immune system. Thus, the patient suffering from this disease has a recurring infection due to the invasion of opportunistic pathogens.

Splenomegaly- Almost all patients suffering from myelofibrosis have enlarged spleen. The increased size of spleen puts pressure on the stomach leading to stomach fullness, pain, and discomfort.

Increased Bleeding- As there is the production of abnormal platelets, these platelets are not able to perform their physiological function. Thus, the patient with myelofibrosis has increased bruising and the increased incidence of bleeding.

Hepatomegaly- Hepatomegaly or enlarged liver is also the symptom of myelofibrosis in some patients. This is due to the extramedullary synthesis of blood cells. This indicates that the blood cells are produced outside the bone marrow. (2)

Conclusion

Age is the primary risk factor for myelofibrosis. Risk increases in people who are above 50 years. Exposure to toxic chemicals and radiations increases the incidences of mutation resulting in abnormal behavior of cells. Other blood disorders such as essential thrombocythemia and polycythemia vera also contribute to developing myelofibrosis.

References

  1. https://www.mayoclinic.org/diseases-conditions/myelofibrosis/symptoms-causes/syc-20355057
  2. https://rarediseases.org/rare-diseases/primary-myelofibrosis/

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Sheetal DeCaria MD

Written, Edited or Reviewed By:

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Last Modified On: May 14, 2019

This article does not provide medical advice. See disclaimer

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