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Diseases

Genetic and Rare Diseases Information Center (GARD)

Unicentric Castleman disease


Other Names for this Disease
  • Localized Castleman disease
  • Unicentric angiofollicular ganglionic hyperplasia
  • Unicentric angiofollicular lymph hyperplasia
Related Diseases
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Your Question

Our daughter-in-law has Castleman's and our granddaughter is now hospitalized with symptoms that are consistent with what we have read about Castleman's. I am asking if it is a hereditary disease.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What causes Castleman disease?

The exact underlying cause of Castleman disease (CD) is poorly understood. However, some scientists suspect that an increased production of interleukin-6 (IL-6) by the immune system may contribute to the development of CD. IL-6 is a substance normally produced by cells within the lymph nodes that helps coordinate the immune response to infection. Increased production of IL-6 may result in an overgrowth of lymphatic cells, leading to many of the signs and symptoms of CD.[1][2]

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 CD, specifically. HHV-8 is found in nearly all people who are HIV-positive and develop multicentric CD, and in up to 60% of affected people without HIV. The HHV-8 virus may possibly cause multicentric CD by making its own IL-6.[1][2]
Last updated: 6/26/2015

Is Castleman disease inherited?

Although the exact underlying cause of Castleman disease is unknown, it is thought to occur sporadically in people with no family history of the condition.[3]
Last updated: 6/26/2015

References
Other Names for this Disease
  • Localized Castleman disease
  • Unicentric angiofollicular ganglionic hyperplasia
  • Unicentric angiofollicular lymph hyperplasia
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.