Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version

Yasufumi Masaki, Hiroshi Kawabata, Kazue Takai, Masaru Kojima, Norifumi Tsukamoto, Yasuhito Ishigaki, Nozomu Kurose, Makoto Ide, Jun Murakami, Kenji Nara, Hiroshi Yamamoto, Yoko Ozawa, Hidekazu Takahashi, Katsuhiro Miura, Tsutomu Miyauchi, Shinichirou Yoshida, Akihito Momoi, Nobuyasu Awano, Soichiro Ikushima, Yasunori Ohta, Natsue Furuta, Shino Fujimoto, Haruka Kawanami, Tomoyuki Sakai, Takafumi Kawanami, Yoshimasa Fujita, Toshihiro Fukushima, Shigeo Nakamura, Tomohiro Kinoshita, Sadao Aoki

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).