Kaieda Shinjiro, Yoshida Naomi, Yamashita Fumiya, Okamoto Masaki, Ida Hiroaki, Hoshino Tomoaki, Fukuda Takaaki
a Department of Medicine , Division of Respirology, Neurology and Rheumatology, Kurume University School of Medicine , Kurume , Japan.
b Center for Rheumatic Diseases, Kurume University Medical Center , Kurume , Japan.
Mod Rheumatol. 2015;25(6):962-6. doi: 10.3109/14397595.2013.844402. Epub 2013 Oct 21.
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is mediated by cytokine overproduction from excessive activation of T lymphocytes and macrophages. We present a dermatomyositis patient with MAS, caused by hypercytokinemia. The combination of tacrolimus and plasma exchange therapy was effective in this case for treating MAS. This combination therapy is especially useful for MAS refractory to steroids.
巨噬细胞活化综合征（MAS），也称为继发性噬血细胞性淋巴组织细胞增生症，是由T淋巴细胞和巨噬细胞过度活化导致细胞因子过度产生所介导的。我们报告了一例由高细胞因子血症引起的伴MAS的皮肌炎患者。在该病例中，他克莫司与血浆置换疗法联合使用对治疗MAS有效。这种联合疗法对难治性类固醇的MAS尤其有用。
