Treatment depends on the type of Castleman disease you have.
Unicentric Castleman disease
Unicentric Castleman disease can be cured by surgically removing the diseased lymph node. If the lymph node is in your chest or abdomen — which is often the case — major surgery may be required.
If surgical removal isn't possible, medication may be used to shrink the lymph node. Radiation therapy also may be an effective way to destroy the affected tissue.
You'll need follow-up exams, including imaging, to check for relapse.
Multicentric Castleman disease
Surgery usually isn't an option for multicentric Castleman disease because of the number of lymph nodes involved. However, surgery to remove an enlarged spleen may be an option to help ease symptoms.
Treatment generally involves medications and other therapies to control cell overgrowth. Specific treatment depends on the extent of your disease and on whether you have HIV or HHV-8 infection or both.
The options include:
Aug. 27, 2014
- Monoclonoal antibodies, to block the action of the IL-6 protein that contributes to cell overgrowth. Your doctor may recommend initial treatment with a monoclonal antibody, such as siltuximab (Sylvant), if you don't have organ damage or HIV or HHV-8 infection.
- Chemotherapy, to slow overgrowth of lymphatic cells. Your doctor may recommend adding chemotherapy if the disease doesn't respond to monoclonal antibodies or if you have organ failure.
- Corticosteroids, to control inflammation.
- Antiviral drugs, to block the activity of HHV-8 or HIV if you have one or both of those viruses.
- Thalidomide (Thalomid), to block the action of the IL-6 protein. Thalidomide is an immune-system modulator that has been shown to be effective at inducing remission in Castleman disease.
- AskMayoExpert. What are the symptoms of Castleman disease? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- Brown JR, et al. Unicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
- Aster JC, et al. Multicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
- Dispenzieri A, et al. The clinical spectrum of Castleman's disease. American Journal of Hematology. 2012;87:997.
- Fajgenbaum DC, et al. HHV-8-negative, idiopathic multicentric Castleman disease: Novel insights into biology, pathogenesis, and therapy. Blood. 2014;123:2924.
- Robinson D, et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: Results from two US treatment centers. British Journal of Haematology. 2014;165:39.
- Talat N, et al. Surgery in Castleman's disease: A systematic review of 404 published cases. Annals of Surgery. 2012;255:677.