2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome
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TAFRO syndrome, first reported in 2010, is a systemic inflammatory disorder manifesting as thrombocytopenia; anasarca, including pleural effusion and ascites; fever; reticulin myelofibrosis and/or renal insufficiency; and organomegaly, including hepatosplenomegaly and lymphadenopathy. The annual incidence rate of TAFRO syndrome in Japan has been estimated to be 0.9–4.9 per million individuals, and the nationwide prevalence to be 860–7240 cases , numbers which are larger than previously expected. Most patients with TAFRO syndrome manifest modest-to-mild systemic lymphadenopathy with characteristic histopathological features resembling those of Castleman disease. Some researchers, therefore, consider that TAFRO syndrome to be a subtype of idiopathic multicentric Castleman disease (iMCD) . However, clinical features of TAFRO syndrome are quite different from those of classical Castleman disease , and we consider TAFRO syndrome to be a distinct clinical entity, although several...
This study was supported in part by grants from the Ministry of Health, Labor and Welfare, Japan (H29 Nanchi, etc. (Nan)-General-019, H27-28 Nanchi, etc. (Nan)-General-002 and -008), and by the Japanese Ministry of Education, Culture, Sports, Science and Technology (Grant No.17591060 and 15K09510), the Kanazawa Medical University Research Foundation (Grant Nos. S2004-16 and S2007-5), Grant for Assist KAKEN from Kanazawa Medical University (Grant No. K2011-7), Grant for Project Research from High-Tech Research Center of Kanazawa Medical University (Grant No. H2011-11) and Grant for Alumni Research(A) from Kanazawa Medical University (AR2012-06).
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Conflict of interest
YM has received research grants from Kyowa Hakko Kirin Pharma, Astellas Pharma, Eisai Pharma, Ono Pharma, Pfizer Pharma, Asahi Kasei Pharma, MSD Pharma, Daiichi-Sankyo Pharma, Taisho Pharma, Taiho Pharma, Takeda Pharma, Chugai Pharma, Teijin Pharma, Nippon Kayaku, and Mochida Pharma, outside the submitted work. All other authors have no conflicts of interest.
- 1.Masaki Y, Kawabata H, Fujimoto S, Kawano M, Iwaki N, Kotani T, et al. Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan. J Clin Exp Hematop. 2019 (in press)Google Scholar