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Genetic and Rare Diseases Information Center (GARD)

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Castleman's disease

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What causes Castleman's disease?

The exact cause of Castleman's disease (CD) is not currently known.[1] Some researchers speculate that problems with the way an affected individual's immune system is working may contribute to the development of CD.[2] Specifically, increased production of interleukin-6 (IL-6) in affected individuals may be involved in the condition's development.[1] IL-6 is a substance normally produced by cells within the lymph nodes; in healthy individuals, IL-6 serves to coordinate the immune response to infection.[1] Increased production of IL-6 contributes to the overgrowth of lymphatic cells and leads to many of the signs and symptoms of CD.[3]

It has also been found that a virus called human herpes virus type 8 (also known as HHV-8, Kaposi's sarcoma-associated herpesvirus, or KSHV) is present in many people with multicentric Castleman's disease. HHV-8 is found in nearly all HIV-positive individuals who develop multicentric CD, and in up to 60% of affected individuals without HIV.[1] Generally, people with unicentric CD are not infected with HHV-8.[3] The HHV-8 virus may possibly cause CD by making its own IL-6. In individuals who are not infected with HHV-8, excess IL-6 production may possibly be caused by a mutation in a gene known as the interleukin 6 promoter.[1][2] More research is needed to better understand the possible cause(s) of the condition.
Last updated: 10/1/2013

  1. Castleman's Disease. NORD. December 8, 2010; Accessed 10/1/2013.
  2. Castleman Disease. American Cancer Society. June 11, 2012; Accessed 10/1/2013.
  3. Castleman disease. Mayo Clinic. September 3, 2011; Accessed 10/1/2013.