This information represents the views of the doctors and nurses serving on the American Cancer Society's Cancer Information Database Editorial Board. These views are based on their interpretation of studies published in medical journals, as well as their own professional experience.

The treatment information in this document is not official policy of the Society and is not intended as medical advice to replace the expertise and judgment of your cancer care team. It is intended to help you and your family make informed decisions, together with your doctor.

Your doctor may have reasons for suggesting a treatment plan different from these general treatment options. Don't hesitate to ask him or her questions about your treatment options.

Surgery

Surgery is often used to obtain a tissue sample to diagnose Castleman disease (CD). A lymph node biopsy is usually a minor procedure.

Surgery also works well to treat localized CD. The type of surgery depends on where the disease is located. When the enlarged lymph node is in a place that is hard to get to, like deep in the chest or abdomen, it means surgery in those areas. Although these are common procedures, they are serious. The patient often has some pain and may need to stay in the hospital for a few days after the operation. If the involved lymph nodes are in a place that is easy to get to, such as in the armpit, then surgery is simpler, there is less pain after the operation, and hospitalization may not be needed.

Radiation therapy

Radiation therapy uses high-energy radiation to kill cells. Radiation focused from a source outside the body is called external beam radiation. Radiation therapy has sometimes been used instead of surgery to treat localized disease.

Side effects of radiation therapy may vary but often include mild skin problems and fatigue. Radiation of the abdomen may cause nausea, diarrhea, and loss of appetite. These side effects tend to improve a short while after the radiation is stopped. When radiation therapy is given to the chest, there is a risk of permanent lung damage leading to breathing problems and shortness of breath. Radiation may also make the side effects of chemotherapy worse if they both are given at the same time.

Corticosteroids

Corticosteroids are a group of drugs related to hormones produced by the adrenal glands. These drugs are useful in treating people with certain immune system diseases and cancers that develop from immune system cells, such as lymphomas. Some patients with multicentric Castleman disease benefit from treatment with these drugs.

Corticosteroids are often taken as pills, but they can also be given as an injection into a vein.. Prednisone, a corticosteroid pill, is often used to treat lymphoma and Castleman disease.

Side effects of corticosteroids can include increased blood sugar that may lead to diabetes, depression, reduced resistance to infections, weakened bones, fatigue, muscle weakness, weight gain, fluid retention, and high blood pressure. Most of these side effects improve after the drug is stopped.

Chemotherapy

Chemotherapy (chemo) is the use of anti-cancer drugs that are injected into a vein or a muscle or are taken by mouth. These drugs enter the bloodstream and reach all areas of the body, making this treatment very useful for multicentric disease. Chemo may be used alone, in combination with corticosteroids, or in combination with radiation therapy (this is called chemoradiation).

Many drugs have been used to treat patients with Castleman disease (CD). Those used most often include carmustine, cladribine, chlorambucil, cyclophosphamide, doxorubicin, etoposide, melphalan, vinblastine, and vincristine. Often several drugs are combined. Chemotherapy combinations like those used for lymphoma have been used. Depending on the drugs, the treatments are given on different schedules but are usually repeated several times in cycles 3 or 4 weeks apart.

Many chemotherapy treatments are given on an outpatient basis (in the doctor's office or clinic), but some require hospital admission. Sometimes a patient takes one drug combination for several cycles and then later is switched to a different one. Because multicentric CD is so rare, there is not a lot of information on which chemo treatment is best or even how well it works.

Chemotherapy drugs can damage normal cells, leading to some side effects. These depend on the type and dose of drugs given and the length of time they are taken. Drugs used in chemotherapy attack cells that are rapidly dividing. This means they will also attack some normal tissues such as the bone marrow, the lining of the mouth and intestines, and the hair follicles, which also grow rapidly to replace cells that wear out. As a result, a patient may have:

• hair loss
• mouth sores
• loss of appetite
• nausea and vomiting
• lowered resistance to infection (due to low white blood cell counts)
• easy bruising and bleeding (due to low platelet counts)
• fatigue and weakness (due to anemia - low red blood cells)

Your doctor will try to avoid or lessen these side effects as much as possible. For example, drugs can be given before or along with chemotherapy to prevent or reduce nausea and vomiting. Most side effects are temporary and go away after treatment is finished.

Because a low white blood cell count is an important risk factor for serious infections, some patients find it helpful to keep track of their counts. If you are interested in this information, ask your doctor or nurse about your blood cell counts and what these numbers mean. You may want to keep a diary of your treatment and blood counts to help you follow the effects of your treatment.

Organs that could be damaged by chemotherapy drugs include the kidneys, liver, testes, ovaries, brain, heart, and lungs. Many of the drugs used to treat Castleman disease (CD) can cause nerve damage, leading to problems such as numbness and tingling in the hands and feet. If serious side effects occur, the chemotherapy may have to be reduced or stopped, at least temporarily. Your doctor will carefully monitor and adjust drug doses because some side effects can be permanent.

Immunotherapy

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 specifically recognizes and 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 oncologist's office or clinic. Side effects are most common during the infusion, and include chills, fever, nausea, rashes, fatigue, and headaches. Unlike regular chemotherapy, rituximab does not cause low blood counts or hair loss.

Immunomodulating agents

Thalidomide: The drug thalidomide is used to treat multiple myeloma, and has helped some patients with CD. Side effects of thalidomide include drowsiness, fatigue, severe constipation, and neuropathy (nerve damage causing pain). There is also an increased 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 can only be obtained through a special program run by the drug company that makes it.

Interferon: Interferon is a hormone-like protein naturally produced by white blood cells to help the immune system fight infections. Some patients with CD disease have improved with interferon treatment. Side effects of this treatment include moderate to severe fatigue, fever, chills, headaches, muscle and joint aches, and mood changes.

Antiviral drugs

Because CD is associated with the virus HHV-8, doctors have had some success in treating a few patients with multicentric CD with drugs that kill this virus. One drug that has been used is called ganciclovir. Many patients with HIV infection are treated with anti-retroviral therapy. The effect of therapy for HIV on CD is not clear.

Last Medical Review: 08/03/2009
Last Revised: 08/03/2009