Successful Treatment with Cyclosporin A in Tocilizumab-resistant TAFRO Syndrome

Yusuke Yamaga; Kiyonobu Tokuyama; Takehiro Kato; Rie Yamada; Masanori Murayama; Tsuneko Ikeda; Noriyoshi Yamakita; Takeshige Kunieda

doi:10.2169/internalmedicine.55.4710

CASE REPORTS

Successful Treatment with Cyclosporin A in Tocilizumab-resistant TAFRO Syndrome

Yusuke Yamaga, Kiyonobu Tokuyama, Takehiro Kato, Rie Yamada, Masanori Murayama, Tsuneko Ikeda, Noriyoshi Yamakita, Takeshige Kunieda

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Keywords: TAFRO syndrome, cyclosporin A, tocilizumab

JOURNAL OPEN ACCESS

2016 Volume 55 Issue 2 Pages 185-190

DOI https://doi.org/10.2169/internalmedicine.55.4710

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Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is a unique clinicopathologic variant of multicentric Castleman's disease that has recently been identified in Japan. Previous reports have shown that affected patients typically respond to immunosuppressive therapy, such as prednisolone and tocilizumab. However, the optimal treatment for refractory TAFRO syndrome, which can be fatal, remains unclear. We herein report a case of tocilizumab-resistant TAFRO syndrome successfully treated with cyclosporin A, indicating that cyclosporine A may be an alternative therapy for refractory TAFRO syndrome.

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