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01 July 2019 : Case report  USA

A Case of Systemic Lupus Erythematosus Presenting as Autoimmune Myelofibrosis

Rare coexistence of disease or pathology

Ikechukwu Mbonu1EF*, Christoph Sossou1E, Christopher B. Nnaoma1E, Xinlai Sun2ADE, Lori Schleicher3D, Wen Xiong4D

DOI: 10.12659/AJCR.916001

Am J Case Rep 2019; 20:937-940


BACKGROUND: Systemic lupus erythematosus (SLE) is characterized by multiorgan involvement and presence of autoantibodies. SLE has a broad range of presentations and manifestations, and as such, its course and organ involvement are unpredictable. The disease results from the interaction of genes, environment, and random effects combining to lead to a loss of tolerance to self-antigens and active autoimmunity. Autoimmune myelofibrosis is a type of non-malignant bone marrow fibrosis that occurs in the presence of systemic autoimmune disease. Cytopenias such as anemia, leukopenia, and thrombocyotopenia are common manifestations of SLE; however, myelofibrosis is a less common and far less recognized complication of SLE.

CASE REPORT: We report a case of a young African American female who presented with severe anemia and leukopenia, subsequently diagnosed with myelofibrosis and then eventually SLE. The identification of myelofibrosis in SLE is critical as it can be a devastating condition when untreated. Fortunately, autoimmune myelofibrosis in SLE is reversible with treatment of the underlying condition.

CONCLUSIONS: Autoimmune myelofibrosis is a rare complication of SLE. Autoimmune myelofibrosis could be the first and only presenting feature of SLE. It is sensible to recognize this relationship, as prompt diagnosis and treatment is crucial. Corticosteroids have been shown to be useful in treating both SLE and the associated autoimmune myelofibrosis.

Keywords: Anemia, Fibrosis, Hematology, Lupus Erythematosus, Systemic, primary myelofibrosis, Rheumatology, Autoimmunity, Diagnosis, Differential, Leukopenia, Thrombocytopenia, young adult

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923