• Hirona Ichimura, Satoshi Ichikawa, Koya Ono, Kyoko Inokura, Yosuke Hoshi, Tsuyoshi Shirai, Noriko Fukuhara, Hisayuki Yokoyama, Hiroshi Fujii, and Hideo Harigae.
• Department of Hematology, Tohoku University Hospital.
• Tohoku J. Exp. Med. 2023 Aug 11; 260 (4): 301304301-304.

AbstractMacrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic diseases, characterized by activated macrophages with hemophagocytosis and multiple organ damage. We report a case of MAS associated with systemic lupus erythematosus that initially presented with severe liver dysfunction. Although it was improved with steroids and plasmapheresis, severe pancytopenia was subsequently experienced, and the bone marrow showed severe aplasia similar to aplastic anemia. Nevertheless, the administration of immunosuppressants resulted in the recovery of blood counts within two weeks. When severe MAS results in cytokine overproduction, bone marrow aplasia may occur, for which immunosuppressive therapy may be highly effective.

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