Severe Bone Marrow Aplasia Following Macrophage Activation Syndrome in Systemic Lupus Erythematosus

Severe Bone Marrow Aplasia Following Macrophage Activation Syndrome in Systemic Lupus Erythematosus

Hirona Ichimura,¹ Satoshi Ichikawa,¹ Koya Ono,¹ Kyoko Inokura,¹ Yosuke Hoshi,² Tsuyoshi Shirai,² Noriko Fukuhara,¹ Hisayuki Yokoyama,¹ Hiroshi Fujii² and Hideo Harigae¹

¹Department of Hematology, Tohoku University Hospital, Sendai, Miyagi, Japan
²Department of Rheumatology, Tohoku University Hospital, Sendai, Miyagi, Japan

Macrophage 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.

Key words —— aplastic anemia; hemophagocytic lymphohistiocytosis; macrophage activation syndrome; severe bone marrow aplasia; systemic lupus erythematosus

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Tohoku J. Exp. Med., 2023 August, 260(4), 301-304.

Correspondence: Satoshi Ichikawa, M.D., Ph.D., Department of Hematology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8574, Japan.

e-mail: satoshi.ichikawa.b4@tohoku.ac.jp