title: Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version.
abstract: TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of multicentric Castleman disease (MCD), other specific features can differentiate between them. Some TAFRO syndrome patients have been successfully treated with glucocorticoids and/or immunosuppressants, including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, and eventually succumb to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The present article reports the 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams. These criteria and classification have been applied and retrospectively validated on clinicopathologic data of 28 patients with this and similar conditions (e.g. MCD with serositis and thrombocytopenia).
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan. yasum@kanazawa-med.ac.jp.
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Masaki
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawabata
authors.affiliation: Division of Hematology, Niigata City General Hospital, Niigata, Japan.
authors.forename: Kazue
authors.initials: K
authors.lastname: Takai
authors.affiliation: Department of Diagnostic Pathology, Dokkyo University School of Medicine, Tochigi, Japan.
authors.forename: Masaru
authors.initials: M
authors.lastname: Kojima
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
authors.forename: Norifumi
authors.initials: N
authors.lastname: Tsukamoto
authors.affiliation: Medical Research Institute, Kanazawa Medical University, Uchinada, Ishikawa, Japan.
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Ishigaki
authors.affiliation: Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Uchinada, Ishikawa, Japan.
authors.forename: Nozomu
authors.initials: N
authors.lastname: Kurose
authors.affiliation: Department of Hematology, Takamatsu Red Cross Hospital, Takamatsu, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Ide
authors.affiliation: Department of Gastroenterology and Hematology, University of Toyama, Toyama, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Murakami
authors.affiliation: Department of Hematology, Seirei Mikatahara General Hospital, Hamamatsu, Shizuoka, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Nara
authors.affiliation: 1st Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamamoto
authors.affiliation: 1st Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Ozawa
authors.affiliation: 1st Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Takahashi
authors.affiliation: Department of Hematology and Rheumatology, Nihon University School of Medicine, Tokyo, Japan.
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Miura
authors.affiliation: Toyama PET Imaging Center, Toyama, Japan.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Miyauchi
authors.affiliation: Department of Hematology, National Hospital Organization Nagasaki Medical Center, Nagasaki, Japan.
authors.forename: Shinichirou
authors.initials: S
authors.lastname: Yoshida
authors.affiliation: Department of Hematology, Niigata Prefectural Central Hospital, Niigata, Japan.
authors.forename: Akihito
authors.initials: A
authors.lastname: Momoi
authors.affiliation: Department of Respiratory Medicine, Japanese Red Cross Medical Center, Tokyo, Japan.
authors.forename: Nobuyasu
authors.initials: N
authors.lastname: Awano
authors.affiliation: Department of Respiratory Medicine, Japanese Red Cross Medical Center, Tokyo, Japan.
authors.forename: Soichiro
authors.initials: S
authors.lastname: Ikushima
authors.affiliation: Department of Pathology, Tokyo University Hospital, Tokyo, Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ohta
authors.affiliation: Department of Internal Medicine, Niigata Cancer Center Hospital, Niigata, Japan.
authors.forename: Natsue
authors.initials: N
authors.lastname: Furuta
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
authors.forename: Shino
authors.initials: S
authors.lastname: Fujimoto
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
authors.forename: Haruka
authors.initials: H
authors.lastname: Kawanami
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Sakai
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kawanami
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
authors.forename: Yoshimasa
authors.initials: Y
authors.lastname: Fujita
authors.affiliation: Division of Hematology and Immunology, Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Fukushima
authors.affiliation: Department of Pathology and Biological Response, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.affiliation: Department of Hematology and Cell Therapy, Aichi Cancer Center Hospital, Nagoya, Aichi, Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kinoshita
authors.affiliation: Department of Pathophysiology, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences, Niigata, Japan.
authors.forename: Sadao
authors.initials: S
authors.lastname: Aoki
mesh_terms: D005871:Castleman Disease / Q000145:classification / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004487:Edema; D005938:Glucocorticoids / Q000627:therapeutic use; D017408:Guidelines as Topic; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013577:Syndrome; D013921:Thrombocytopenia
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: Anasarca; Cyclosporin A; Glucocorticoid; Thrombocytopenia; Tocilizumab