TAFRO Syndrome: A Disease Requiring Immediate Medical Attention

Yasufumi Masaki; Yusuke Ueda; Hiroto Yanagisawa; Kotaro Arita; Tomoyuki Sakai; Kazunori Yamada; Shuichi Mizuta; Toshihiro Fukushima; Kazue Takai; Sadao Aoki; Hiroshi Kawabata

doi:10.2169/internalmedicine.9622-22

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TAFRO Syndrome: A Disease Requiring Immediate Medical Attention

Yasufumi Masaki, Yusuke Ueda, Hiroto Yanagisawa, Kotaro Arita, Tomoyuki Sakai, Kazunori Yamada, Shuichi Mizuta, Toshihiro Fukushima, Kazue Takai, Sadao Aoki, Hiroshi Kawabata

Author information

Yasufumi Masaki
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Yusuke Ueda
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Hiroto Yanagisawa
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Kotaro Arita
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Tomoyuki Sakai
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Kazunori Yamada
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Shuichi Mizuta
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Toshihiro Fukushima
Department of Hematology and Immunology, Medicine, Kanazawa Medical University, Japan
Kazue Takai
Department of Hematology, Niigata City General Hospital, Japan
Sadao Aoki
Department of Pathophysiology, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences, Japan
Hiroshi Kawabata
Department of Hematology, National Hospital Organization Kyoto Medical Center, Japan

Keywords: idiopathic multicentric Castleman disease, POEMS syndrome, interleukin-6, tocilizumab, rituximab, cyclosporin A

JOURNAL OPEN ACCESS

2023 Volume 62 Issue 1 Pages 27-32

DOI https://doi.org/10.2169/internalmedicine.9622-22

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Abstract

TAFRO syndrome was first described in 2010, standing for thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly. Because the lymph node histopathology of TAFRO syndrome mimics idiopathic multicentric Castleman disease (iMCD), some researchers consider TAFRO syndrome to be a subtype of iMCD. However, the clinical features of TAFRO syndrome considerably differ from those of iMCD without TAFRO. The clinical features of patients with TAFRO syndrome with or without iMCD-histopathology are similar, and these patients require an accurate diagnosis and urgent treatment. Although a histological diagnosis, including a differential diagnosis, is important, lymph node involvement in patients with TAFRO syndrome is usually modest or sometimes absent. Furthermore, a bleeding tendency due to thrombocytopenia and severe anasarca hampers performing a biopsy. Nonetheless, patients with various other disorders may manifest TAFRO syndrome-like symptoms, making the differential diagnosis in borderline cases difficult. Therefore, the establishment of precise and specific biomarkers is important.

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