Castleman Disease

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Treating Castleman Disease TOPICS

Immunotherapy for Castleman disease

Immunotherapy is treatment that either boosts the patient's own immune system or uses man-made versions of the normal parts of the immune system.

Monoclonal antibodies

Monoclonal antibodies are special immune proteins made in the lab. They are directed toward specific molecules on the surface of cells.

Rituximab (Rituxan®) is a monoclonal antibody that is widely used for lymphoma. It can also be helpful in treating Castleman disease (CD). Rituximab attaches to a protein called CD20 that is found on the surface of some lymphocytes. This attachment tells the cell to die.

Patients get rituximab through infusion into a vein (IV) at the doctor's office or clinic. As when it is used to treat lymphoma, it is often given along with chemotherapy.

Side effects of rituximab are most common during the infusion, and can include chills, fever, nausea, rashes, fatigue, and headaches. Rarely, more severe side effects occur during the infusion, such as trouble breathing and low blood pressure. This drug may also increase a person's risk of certain infections. Unlike regular chemotherapy, rituximab does not cause low blood counts or hair loss.

In people who have ever been infected with the hepatitis B virus, this drug can sometimes cause the infection to become active again. Your doctor may check your blood for signs of a prior hepatitis infection before starting this drug to see if it is safe.

Newer antibodies that attack other targets are also being studied for use against CD. These are discussed in the section, “What’s new in research and treatment of Castleman disease?


The drug thalidomide (Thalomid®) is a type of drug called an immunomodulating agent. It is thought to work by affecting parts of a person's immune system, although it’s not exactly clear how it does this. It is used to treat certain cancers of immune cells such as multiple myeloma and some types of lymphoma, but it has also helped some patients with CD.

Thalidomide is taken once a day as a capsule. Side effects can include drowsiness, fatigue, severe constipation, low white blood cell counts (with an increased risk of infection), and neuropathy (nerve damage causing pain). It also increases the risk of serious blood clots that start in the leg and can travel to the lungs. Because thalidomide causes severe birth defects if taken during pregnancy, this drug should not be used by women who are or may become pregnant.


Interferon is a hormone-like protein naturally made by white blood cells in the body to help the immune system fight infections. Some patients with CD have improved with man-made interferon treatment.

Interferon is given by an injection, either daily or several times a week. This may be into a vein (IV), under the skin (SubQ), or into a muscle (IM). It may be given in a doctor's office, or you or a family member can be taught how to give the medicine under the skin.

Side effects of this treatment can include fatigue, fever, chills, headaches, muscle and joint aches, and mood changes. Because of these side effects, interferon is not used very often. It may be given to some patients in addition to chemotherapy.

Last Medical Review: 06/11/2012
Last Revised: 06/11/2012