Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan

Authors:
  • HKHiroshi Kawabata
    Hiroshi Kawabata
  • SFShino Fujimoto
    Shino Fujimoto
  • MKMitsuhiro Kawano
    Mitsuhiro Kawano
  • NINoriko Iwaki
    Noriko Iwaki
  • TKTakeharu Kotani
    Takeharu Kotani
  • ANAkikatsu Nakashima
    Akikatsu Nakashima
  • NKNozomu Kurose
    Nozomu Kurose
  • KTKazue Takai
    Kazue Takai
  • SASadao Aoki
    Sadao Aoki
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Abstract
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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