Aplastic anemia is a bone marrow failure syndrome characterized by peripheral
cytopenias and hypocellular bone marrow. Although aplastic anemia is idiopathic in
most cases, rheumatic diseases such as systemic lupus erythematosus are recognized as
causes of aplastic anemia, with their possible etiological mechanisms being T and B
lymphocyte dysfunction and pro-inflammatory cytokines and autoantibody production
directed against bone marrow components. In the course of the human immunodeficiency
virus infection/acquired immunodeficiency syndrome, the identification of
autoantibodies and the occurrence of rheumatic events, in addition to the natural
course of systemic lupus erythematosus which is modified by immune changes that are
characteristic of human immunodeficiency virus infection/acquired immunodeficiency
syndrome, make the diagnosis of systemic lupus erythematosus challenging. This study
reports the case of a woman with acquired immunodeficiency syndrome treated with a
highly active antiretroviral therapy, who had prolonged cytopenias and hypocellular
bone marrow consistent with aplastic anemia. The clinical picture, high
autoantibodies titers, and sustained remission of the patient's hematological status
through immunosuppression supported the diagnosis of systemic lupus
erythematosus-associated aplastic anemia. This is the first report of aplastic anemia
concurrent with systemic lupus erythematosus and acquired immunodeficiency syndrome,
providing additional evidence that immune dysfunction is a key part of the
pathophysiological mechanism of aplastic anemia.
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