Aplastic anemia associated to systemic lupus erythematosus in an AIDS patient: a case report

Abstract

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.

Keywords: Acquired immunodeficiency syndrome; Anemia, aplastic; Autoantibodies; Case reports; Female; HIV infections; Humans; Lupus erythematosus, systemic; Pancytopenia.

Figure 1

Hypocellular bone marrow marked by intense replacement of normal cellularity by adipose tissue. Maximow staining, 200 x original magnification

Figure 2

Hypocellular bone marrow with 10% hematopoietic cells, predominance of erythroid lineage cells, and abundant hemosiderin deposits (3+/4+). Hematoxylin and eosin staining, 400 x original magnification