Causes of Castleman's Disease

The underlying cause of Castleman’s Disease (CD) is unknown.1 It frequently occurs in patients with human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8) infections, but also occurs in noninfected patients.2

The underlying cause of Castleman’s Disease (CD) is unknown.1 It frequently occurs in patients with human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8) infections, but also occurs in noninfected patients.2

Evidence shows that interleukin-6 (IL-6), an inflammatory mediator, has a pivotal role in the pathology of the disease. IL-6 levels are notably increased in patients with CD. IL-6 may contribute to the disease in a number of ways, including3:

  • Stimulation of increased B-cell production, which is in part responsible for lymph node hyperplasia and enlargement
  • Increased secretion of vascular endothelial growth factor (VEGF), leading to increased blood vessel formation in the lymph nodes
  • Immune function dysregulation, resulting in autoimmune phenomena
  • Pro-inflammatory effects, which may contribute to systemic symptoms

Additional contributing factors, including certain oncogenic proteins, epithelial growth factor receptor (EGFR) expression, and interleukin-1, have been explored, but their pathogenic roles in CD are not well-defined.1

This short video will provide additional information about the mechanism of disease.

  1. Roca B. Castleman’s disease: a condition with protean manifestations. Onkologie. 2011;34(8-9):412-414.
  2. van Rhee F, Stone K, Szmania S, Barlogie B, Singh Z. Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy. Clin Adv Hematol Oncol. 2010;8(7):486-498.
  3. El-Osta HE, Kurzrock R. Castleman’s disease: from basic mechanisms to molecular therapeutics. Oncologist. 2011;16(4):497-511.

This short video will provide additional information about the mechanism of disease.