ABSTRACT
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.
ABSTRACT
A 55-year-old man was diagnosed with therapy-related chronic myelomonocytic leukemia (t-CMML) after exposure to tegafur/gimeracil/oteracil. Although he was refractory to hydroxyurea and low-dose cytarabine, combination therapy with cytarabine, aclarubicin and azacitidine (CA-AZA) provided good disease control, and he underwent allogeneic stem cell transplantation. This report has two key massages. First, tegafur/gimeracil/oteracil may have a potential risk of developing t-CMML. Second, CA-AZA therapy may be considered as a therapeutic option for patients with t-CMML.
