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Treatments and drugs
By Mayo Clinic staffTreatment depends largely on the type of Castleman disease you have. Treatment for unicentric Castleman disease is almost always with surgery, while multicentric Castleman disease requires more systemic therapies.
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 with unicentric disease — major surgery may be required to remove the node. This usually involves a hospital stay. If the affected node is in a place that's easy to access, such as in your underarm area or your neck, a simpler procedure may be performed that doesn't require hospitalization.
If surgical removal isn't possible, such as if the lymph node is difficult to get to, radiation therapy may be an effective way to destroy the affected tissue.
Multicentric Castleman disease
Treating multicentric Castleman disease is generally more difficult. Because the disease is rare, has varied nonspecific signs and symptoms, and spontaneously goes into remission at times, doctors have found it difficult to identify the best treatment. In addition, there are no clinical trials that offer definitive evidence in favor of any therapy. It's also not clear if treatment should differ based on HIV status.
Most therapies are palliative, which means their goal is to relieve signs and symptoms rather than cure the illness. Surgery usually isn't an option for multicentric disease because of the number of lymph nodes involved, although sometimes removing an enlarged spleen may help ease symptoms.
Therapies that are commonly used for multicentric disease, with varying degrees of success, include the following. Doctors generally try as many therapies as they can to provide the most relief.
- Corticosteroids. The basic function of corticosteroid therapy is to control inflammation. With multicentric Castleman disease, doctors usually prescribe steroids, such as prednisone, in combination with other therapies. Long-term use of steroids may reduce your resistance to infection, as well as increase your blood pressure, cause weight gain and weaken your bones.
- Chemotherapy. Anti-cancer drugs (chemotherapy) can be useful because they target rapidly reproducing cells, such as those associated with Castleman disease. If chemotherapy is stopped, though, symptoms tend to return and further treatment is needed to maintain symptom relief. Still, chemotherapy has helped the disease go into remission for some people. Usually, doctors use a combination of anti-cancer drugs. Side effects may include hair loss, nausea, vomiting, loss of appetite and reduced resistance to infection.
- Antiviral drugs. Antiviral medications, such as ganciclovir, are known to inhibit the activity of the virus HHV8. The success of this therapy has been mixed — effective for some people, but not for others. Doctors still have many questions about antiviral therapy for Castleman disease, such as which antiviral is best and when the best time to administer it is.
- Monoclonal antibodies. These man-made antibodies are designed to zero in on specific cells in the body that aren't working right. One such antibody that may be effective for multicentric Castleman disease works by neutralizing or blocking the activity of IL-6, the immune-cell protein suspected of playing a role in causing the disease. Another monoclonal antibody that may help is rituximab (Rituxan), a drug that targets a different type of cell associated with Castleman disease.
- Immune modulators. Doctors have tried other drugs that help to regulate the immune system (immune modulators) in the hopes that these drugs might have some effect. Interferon alpha has both immunoregulatory and antiviral effects and has been used successfully in a few people with Castleman disease, although symptoms usually return once you stop taking the drug. Thalidomide, a powerful immunomodulatory drug, also has helped a few people and may help decrease the production of IL-6.
- Castleman disease (Formerly Castleman's disease). American Cancer Society. http://documents.cancer.org/6636.00/6636.00.pdf. Accessed July 17, 2009.
- Aster JC, et al. Castleman's disease. http://www.uptodate.com/home/index.html. Accessed July 10, 2009.
- What is Castlemans disease. International Castleman's Disease Organization. http://www.castlemans.org/index.php?option=com_content&view=article&id=19&Itemid=27. Accessed July 17, 2009.
- Dham A, et al. Castleman disease. Current Opinion in Hematology. 2007;14:359.
- Pica F, et al. Transmission of human herpesvirus 8: An update. Current Opinion in Infectious Diseases. 2007;20:152.
- Dispenzieri A, et al. Treatment of Castleman's Disease, Current Treatment Options in Oncology. 2005;6:255.
