Recent Advances in Castleman Disease

Oncol Res Treat. 2022;45(11):693-704. doi: 10.1159/000526640. Epub 2022 Oct 11.


Background: Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD).

Summary: During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD.

Key messages: We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease.

Keywords: Castleman disease; HHV-8; Multicentric CD; Siltuximab; Unicentric CD.

Publication types

  • Review

MeSH terms

  • Castleman Disease* / drug therapy
  • Castleman Disease* / therapy
  • Herpesvirus 8, Human*
  • Humans
  • Interleukin-6 / therapeutic use
  • Signal Transduction


  • Interleukin-6

Supplementary concepts

  • Multi-centric Castleman's Disease