Journal of Clinical and Experimental Hematopathology
Online ISSN : 1880-9952
Print ISSN : 1346-4280
ISSN-L : 1346-4280
Original Articles
Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan
Yasufumi MasakiHiroshi KawabataShino FujimotoMitsuhiro KawanoNoriko IwakiTakeharu KotaniAkikatsu NakashimaNozomu KuroseKazue TakaiRitsuro SuzukiSadao Aoki
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2019 Volume 59 Issue 4 Pages 175-178


Castleman disease is a polyclonal lymphoproliferative disease which is clinically classified into unicentric (UCD) and multicentric (MCD). TAFRO syndrome is a relatively new concept that partly overlaps with MCD. Due to their rarity, their incidence remains unknown. This study investigated the incidence and prevalence of UCD, MCD, and TAFRO syndrome in Japan using a fixed-point observation method based on their incidence in Ishikawa prefecture. The annual incidences of MCD, UCD, and TAFRO syndrome in Japan were 309-731, 71-542, and 110-502, respectively, yielding annual incidence rates per million individuals of 2.4-5.8, 0.6-4.3, and 0.9-4.9, respectively, and nationwide prevalence of 4,180-14,900, 1,350-10,300, and 860-7,240, respectively. In conclusion, MCD, UCD and TAFRO syndrome may not be as rare as previously estimated in Japan.

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© 2019 by The Japanese Society for Lymphoreticular Tissue Research

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