Treatments and drugs

By Mayo Clinic Staff

Treatment 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, medications, such as the monoclonal antibody rituximab (Rituxan) or corticosteroids, may be used to shrink the lymph node. Radiation therapy may also 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.

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.

Medications are commonly used for multicentric disease, with varying degrees of success. Doctors generally try as many treatments as they can to provide the most relief. Therapies include the following:

  • 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. Although there haven't been any definitive clinical trials, several small studies have found that rituximab can cause complete remission in some people. Treatment with this medication has been promising enough that it's generally considered the first treatment to try. Side effects may include fever, headache, fatigue, skin rash and nausea.
  • 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 HHV-8. 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 is the best time to administer it.
  • 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 (Thalomid), a powerful immunomodulatory drug, also has helped a few people and may help decrease the production of IL-6.
Sep. 03, 2011