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

Multicentric Castleman Disease

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How might multicentric Castleman's disease be treated?
Treatment of multicentric Castleman disease (MCD) is challenging, and no single treatment works for all people with the disease.[1] Treatment options may depend on the type of MCD (HHV-8-associated or idiopathic) as well as the severity of symptoms.

HHV-8-associated MCD is typically initially treated with rituximab to fight against immune cells called B lymphocytes. This treatment is reportedly highly effective for HHV-8-associated MCD. Antiviral medications (particularly for those with HIV) and/or chemotherapy may also be recommended.[2]

Idiopathic MCD (iMCD) is typically initially treated with siltuximab or tocilizumab (which aim to control activity of interleukin-6) with or without corticosteroids. Unfortunately, about half of people do not improve with these therapies. In those with only mild symptoms, rituximab may be an alternative option for initial treatment. In very severe cases, adjuvant combination chemotherapy is also recommended. Other types of therapies may be recommended if the above therapies are not effective.[3]
Last updated: 11/6/2018

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  1. Treatment of Multicentric Castleman Disease. American Cancer Society. January 12, 2017; https://www.cancer.org/cancer/castleman-disease/treating.html.
  2. Castleman Disease. National Organization for Rare Disorders (NORD). 2017; https://rarediseases.org/rare-diseases/castlemans-disease/.
  3. van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. September 4, 2018; [Epub ahead of print]:https://www.ncbi.nlm.nih.gov/pubmed/30181172.