Epidemiology of Castleman Disease

Hematol Oncol Clin North Am. 2018 Feb;32(1):1-10. doi: 10.1016/j.hoc.2017.09.001.

Abstract

Castleman disease is a rare entity, including unicentric Castleman disease (UCD), human herpesvirus-8 plus Castleman disease (HHV-8+MCD), and idiopathic multicentric Castleman disease (iMCD). UCD is the most common at 16 per million person years and occurs at every age. HHV-8+MCD incidence varies widely, mostly affecting human immunodeficiency virus-positive men. iMCD is likely a more heterogeneous disease with an estimated incidence of 5 per million person years. Improved definitions should improve understanding of the epidemiology of Castleman disease and its subtypes.

Keywords: HHV-8; Multicentric Castleman; TAFRO; Unicentric Castleman.

Publication types

  • Review

MeSH terms

  • Castleman Disease / classification*
  • Castleman Disease / epidemiology*
  • Female
  • HIV Seropositivity / complications
  • HIV Seropositivity / epidemiology
  • Herpesviridae Infections / classification
  • Herpesviridae Infections / epidemiology
  • Herpesvirus 8, Human
  • Humans
  • Incidence
  • Male
  • Sex Factors