It's not clear what causes Castleman disease. However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease.
The HHV-8 virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor of the blood vessel walls that can be a complication of HIV/AIDS. Studies have found that HHV-8 is present in HIV-positive people who have Castleman disease, and in 40 to 50 percent of HIV-negative people with Castleman disease.
The precise role of HHV-8 is unclear. But it appears to cause malfunctioning immune system cells to reproduce rapidly. The immune system cells produce a protein called interleukin-6 (IL-6) that contributes to the overgrowth of lymphatic cells.
Aug. 27, 2014
- AskMayoExpert. What are the symptoms of Castleman disease? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- Brown JR, et al. Unicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
- Aster JC, et al. Multicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
- Dispenzieri A, et al. The clinical spectrum of Castleman's disease. American Journal of Hematology. 2012;87:997.
- Fajgenbaum DC, et al. HHV-8-negative, idiopathic multicentric Castleman disease: Novel insights into biology, pathogenesis, and therapy. Blood. 2014;123:2924.
- Robinson D, et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: Results from two US treatment centers. British Journal of Haematology. 2014;165:39.
- Talat N, et al. Surgery in Castleman's disease: A systematic review of 404 published cases. Annals of Surgery. 2012;255:677.
You Are ... The Campaign for Mayo Clinic
Mayo Clinic is a not-for-profit organization. Make a difference today.