Autoimmune myelofibrosis (AIMF)

 

Overview

A form of non-cancerous myelofibrosis occurring with an autoimmune disease. Myelofibrosis occurs when abnormal blood cells and fibers build up in the bone marrow, causing severe anemia. If the condition is associated with a well-established autoimmune disease like systemic lupus erythematosus, it is referred to as secondary AIMF. On the other hand, primary AIMF refers to cases in which autoantibodies are present without a well-established autoimmune disorder.

Common Symptoms

The scarring in bone marrow caused by myelofibrosis results in anemia and thrombocytopenia. Symptoms include weakness, fatigue, and excessive bleeding.  Patients with secondary AIMF will also experience symptoms associated with their coexisting autoimmune disease.

Coexisting Diseases and Conditions

Systemic lupus erythematosus and rheumatoid arthritis.

Risk Factors and Prevalence

While risk factors for myelofibrosis include older age and chemical or radiation exposures, no specific risk factors have been identified with respect to AIMF.

Sources

  1. Article Sources and Footnotes
    1. Mayo Clinic Staff. (2021, June 8). Myelofibrosis. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/myelofibrosis/symptoms-causes/syc-20355057

    2. Piatek, C. I., Vergara-Lluri, M. E., Pullarkat, V., Siddiqi, I. N., O’Connell, C., Brynes, R. K., & Feinstein, D. I. (2017). Autoimmune Myelofibrosis: Clinical Features, Course, and Outcome. Acta Haematologica, 138(3), 129–137. https://doi.org/10.1159/000479103

    3. Pundole, X., Konoplev, S., Oo, T. H., & Lu, H. (2015). Autoimmune Myelofibrosis and Systemic Lupus Erythematosus in a Middle-Aged Male Presenting Only with Severe Anemia: A case report. Medicine, 94(19), e741. https://doi.org/10.1097/MD.0000000000000741

    4. Vergara-Lluri, M. E., Piatek, C. I., Pullarkat, V., Siddiqi, I. N., O’Connell, C., Feinstein, D. I., & Brynes, R. K. (2014). Autoimmune myelofibrosis: An update on morphologic features in 29 cases and review of the literature. Human Pathology, 45(11), 2183–2191. https://doi.org/10.1016/j.humpath.2014.07.017

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