Castleman disease

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.

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.

Last updated on 05-01-20

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.

Last updated on 05-01-20

Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: Url:
Name: Castleman Disease Collaborative Network P.O. Box 3614
Paso Robles, CA, 93447,
Phone: +1-610-304-0696 (for patients and families) Email: Url: Office phone: +1-215-614-0935
Aster JC, Brown JR & Munshi NC. Multicentric Castleman's disease UpToDate. Jan 05, 2015; Reference Link

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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