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Genetic and Rare Diseases Information Center (GARD)

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Castleman's disease

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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.

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.[1] 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

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