National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Castleman disease

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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.

The following information may help to address your question:


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

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  1. Neetu Radhakrishnan, MD. Castleman Disease. Medscape Reference. January 2015; http://emedicine.medscape.com/article/2219018-overview.
  2. Jon C Aster, MD; Jennifer R Brown, MD, PhD; Nikhil C Munshi, MD. Multicentric Castleman's disease. UpToDate. January 2015; Accessed 6/25/2015.
  3. Castleman disease. Orphanet. January 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=160.