Castleman disease

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Causes

By Mayo Clinic staff

The exact cause of Castleman disease remains unknown. Infection by a virus called the human herpes virus 8 (HHV8) is associated with Castleman disease, and doctors suspect that it may play a causal 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 sarcoma.

Researchers aren't clear what the precise role of HHV8 may be, though it appears that it may help malfunctioning immune system cells reproduce rapidly. The immune system cells are called interleukin-6 (IL-6), and a protein produced by these cells may contribute to the overgrowth of lymphatic cells.

The HHV virus is likely transmitted through saliva, but may also be passed from person to person via sexual contact, blood transfusions and transplants.

Generally, unicentric Castleman disease isn't associated with HHV8.

References
  1. Castleman disease (Formerly Castleman's disease). American Cancer Society. http://documents.cancer.org/6636.00/6636.00.pdf. Accessed July 17, 2009.
  2. Aster JC, et al. Castleman's disease. http://www.uptodate.com/home/index.html. Accessed July 10, 2009.
  3. What is Castlemans disease. International Castleman's Disease Organization. http://www.castlemans.org/index.php?option=com_content&view=article&id=19&Itemid=27. Accessed July 17, 2009.
  4. Dham A, et al. Castleman disease. Current Opinion in Hematology. 2007;14:359.
  5. Pica F, et al. Transmission of human herpesvirus 8: An update. Current Opinion in Infectious Diseases. 2007;20:152.
  6. Dispenzieri A, et al. Treatment of Castleman's Disease, Current Treatment Options in Oncology. 2005;6:255.

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Sept. 5, 2009

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