Castleman disease

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Causes

By Mayo Clinic staff

It's not clear what causes Castleman disease. Infection by a virus called the human herpesvirus 8 (HHV-8) is associated with Castleman disease, and doctors suspect that it may play a role, especially in multicentric Castleman disease. This virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor of the blood vessel walls, which is also common in people with multicentric Castleman disease. People who are HIV-positive are more likely to have both Castleman disease and Kaposi's sarcoma.

Researchers aren't clear what the precise role of HHV-8 may be, though it appears that it may 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 and leads to many of the signs and symptoms of Castleman disease.

Generally, people with unicentric Castleman disease aren't infected with HHV-8.

References
  1. Aster JC, et al. Castleman's disease. http://www.uptodate.com/home/index.html. Accessed July 19, 2011.
  2. Castleman disease. American Cancer Society. http://www.cancer.org/acs/groups/cid/documents/webcontent/003093-pdf.pdf. Accessed July 19, 2011.
  3. About Castleman's disease. International Castleman's Disease Organization. http://www.castlemans.org/ICDO_booklet_v3.pdf. Accessed July 19, 2011.
  4. Pica F, et al. Transmission of human herpesvirus 8: An update. Current Opinion in Infectious Diseases. 2007;20:152.
  5. Bandera B, et al. Treatment of unicentric Castleman disease with neoadjuvant rituximab. Chest. 2010;138:1239.
  6. Reddy D, et al. HIV-associated multicentric Castleman disease. Current Opinion in Oncology. In press. Accessed [month day, 2011]. Accessed July 19, 2011.
DS01000 Sept. 3, 2011

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