title: Proposed diagnostic criteria, disease severity classification, and treatment strategy for a novel disorder; TAFRO syndrome.
abstract: TAFRO syndrome is a systemic inflammatory disorder manifesting as 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 remains unknown. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of Castleman's disease, other specific features can differentiate between the two. Some patients have been successfully treated with glucocorticoids and/or immunosuppressants including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, eventually succumbing to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams, are presented herein. Furthermore, clinicopathological data on 28 patients with this condition and similar symptoms (e.g., MCD with serositis and thrombocytopenia) were analyzed retrospectively.
authors: Hematology and Immunology, Medicine, Kanazawa Medical University.
authors: Yasufumi
authors: Y
authors: Masaki
authors: Hiroshi
authors: H
authors: Kawabata
authors: Kazue
authors: K
authors: Takai
authors: Norifumi
authors: N
authors: Tsukamoto
authors: Shino
authors: S
authors: Fujimoto
authors: Yasuhito
authors: Y
authors: Ishigaki
authors: Nozomu
authors: N
authors: Kurose
authors: Masaru
authors: M
authors: Kojima
authors: Shigeo
authors: S
authors: Nakamura
authors: Tomohiro
authors: T
authors: Kinoshita
authors: Sadao
authors: S
authors: Aoki
mesh_terms: D004487:Edema / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D005334:Fever / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D005355:Fibrosis / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D012155:Reticulin / Q000737:chemistry; D012720:Severity of Illness Index; D013921:Thrombocytopenia / Q000150:complications / Q000175:diagnosis / Q000628:therapy
chemical_list: D012155:Reticulin