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

Yasufumi Masaki, Hiroshi Kawabata, Shino Fujimoto

    Journal of Clinical and Experimental Hematopathology : JCEH
    |November 12, 2019
    PubMed

    Insights

    Castleman disease (UCD, MCD) and TAFRO syndrome may be more common in Japan than previously thought. This study estimated their incidence and prevalence, suggesting they are not as rare as once believed.

    Area of Science:

    • Hematology
    • Oncology
    • Epidemiology

    Background:

    • Castleman disease, a polyclonal lymphoproliferative disorder, is classified into unicentric (UCD) and multicentric (MCD) forms.
    • TAFRO syndrome is a recently described condition with clinical overlap with MCD.
    • The true incidence and prevalence of these rare conditions remain largely unknown.

    Purpose of the Study:

    • To investigate the incidence and prevalence of UCD, MCD, and TAFRO syndrome in Japan.
    • To provide updated epidemiological data for these rare lymphoproliferative disorders.

    Main Methods:

    • A fixed-point observation method was employed.
    • Data were collected based on the incidence observed in Ishikawa prefecture, Japan.
    • Calculations were extrapolated to estimate nationwide incidence and prevalence.

    Main Results:

    • Estimated annual incidences per million individuals: MCD (2.4-5.8), UCD (0.6-4.3), and TAFRO syndrome (0.9-4.9).
    • Estimated nationwide prevalence ranges: MCD (4,180-14,900), UCD (1,350-10,300), and TAFRO syndrome (860-7,240).
    • These findings suggest higher occurrence rates than previously assumed.

    Conclusions:

    • Castleman disease (UCD, MCD) and TAFRO syndrome may not be as rare in Japan as previously estimated.
    • The study provides crucial epidemiological insights into these rare hematologic conditions.
    Keywords:
    Statistical Research Committee of Japanese Society of Hematologydisease incidencefixed-point observation methodspecific use survey

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