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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Castleman's disease


Other Names for this Disease

  • Angiofollicular ganglionic hyperplasia
  • Angiofollicular lymph hyperplasia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Castleman's disease?

What causes Castleman's disease?

Is Castleman's disease inherited?

What is Castleman's disease?

Castleman’s disease is a lymphoproliferative disorder affecting the lymph nodes and related tissues. While the cause of Castleman's disease is unknown, many doctors suspect a virus is involved. Problems with the way an individual's immune system functions may also contribute to the development of the condition. There are 2 main forms of Castleman's disease: localized (discussed here) and multicentric. They affect people very differently. Localized or unicentric Castleman's disease only affects a single set of lymph nodes and is not widespread. The lymph nodes that are more commonly affected are in the chest and abdomen. Castleman’s disease causes the lymph nodes to get larger. The enlarged lymph nodes press on other organs and tissues inside the chest or abdomen, causing discomfort or difficulty breathing. Sometimes the enlarged lymph nodes are in places such as the neck, groin, or armpit and can be easily felt. People with localized Castleman's disease are often cured when the lymph node is removed with surgery. Chemotherapy and radiation may also be used.[1]
Last updated: 4/25/2012

What causes Castleman's disease?

The exact cause of Castleman's disease (CD) is not currently known.[2] Some researchers speculate that problems with the way an affected individual's immune system is working may contribute to the development of CD.[3] Specifically, increased production of interleukin-6 (IL-6) in affected individuals may be involved in the condition's development.[2] 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.[2] Increased production of IL-6 contributes to the overgrowth of lymphatic cells and leads to many of the signs and symptoms of CD.[4]

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.[2] Generally, people with unicentric CD are not infected with HHV-8.[4] 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.[2][3] More research is needed to better understand the possible cause(s) of the condition.
Last updated: 10/1/2013

Is Castleman's disease inherited?

Castleman's disease is thought to be sporadic. This means that it is thought to occur by chance in individuals who have no history of the condition in the family, and is not likely to recur in a family.[5] We were unable to find reports of any familial cases of Castleman's disease in the available medical literature. It has been proposed that in affected individuals who are not infected with human herpes virus 8 (HHV-8), excess production of interleukin 6 (IL-6) may be caused by a mutation in a gene known as the interleukin 6 promoter.[2] However, more research is needed to clarify this information.
Last updated: 10/1/2013

References
  1. Detailed Guide: Castleman Disease. American Cancer Society. 2011; http://www.cancer.org/Cancer/CastlemanDisease/DetailedGuide/. Accessed 8/18/2011.
  2. Castleman's Disease. NORD. December 8, 2010; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/532/viewAbstract. Accessed 10/1/2013.
  3. Castleman Disease. American Cancer Society. June 11, 2012; http://www.cancer.org/Cancer/CastlemanDisease/DetailedGuide/castleman-disease-what-causes. Accessed 10/1/2013.
  4. Castleman disease. Mayo Clinic. September 3, 2011; http://www.mayoclinic.com/health/castleman-disease/DS01000/DSECTION=causes. Accessed 10/1/2013.
  5. Françoise SARROT-REYNAULD. Castleman disease. Orphanet. November 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=160. Accessed 10/1/2013.


Other Names for this Disease
  • Angiofollicular ganglionic hyperplasia
  • Angiofollicular lymph hyperplasia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.