How Is Castleman Disease Treated
Surgery
Surgery is often used to obtain a tissue sample to diagnose Castleman disease. It is usually a minor procedure to biopsy a lymph node.
Surgery is also a very effective treatment for localized disease. The type of surgery depends on where the disease is located. It may be in a place that is hard to get to, like the center of the chest or abdomen. The surgeon would then have to cut into the chest or abdomen. These are common procedures, but they can cause pain and people will need to be in the hospital for a few days after the operation. If the involved lymph nodes are 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 effectively treat localized disease.
Side effects of radiation therapy may include mild skin problems or fatigue. Radiation of the abdomen may cause nausea and diarrhea. Often these go away after a short while. Chest radiation therapy may cause lung damage and lead to breathing problems and shortness of breath. Radiation may also make the side effects of chemotherapy worse.
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 taken as pills. Prednisone is the corticosteroid drug most commonly used in these conditions.
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.
Chemotherapy
Chemotherapy is the use of anticancer 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. Chemotherapy may be used alone, in combination with corticosteroids, or in combination with radiation therapy (chemoradiation).
Many drugs may be used to treat patients with Castleman disease. Those used most often include cyclophosphamide, chlorambucil, melphalan, doxorubicin, vincristine, vinblastine, carmustine, and etoposide. Often several drugs are combined. Depending on the drugs, the treatments are given on different schedules but are usually repeated several times in cycles 3 or 4 weeks apart. Recently, a study reported a good response to the drug 2-chloro-deoxyadenosine (2CdA) in 2 of 3 patients.
Many chemotherapy treatments are given on an outpatient basis (in the doctor’s office or clinic), but some require hospital admission. Sometimes a patient may take one chemotherapy combination for several cycles and later be switched to a different one. Because multicentric Castleman disease is so rare, there is not a lot of information about the effectiveness of chemotherapy.
Because chemotherapy drugs can damage normal cells, some side effects may occur. 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)
These side effects are usually temporary and go away after treatment is finished. Still, doctors try to avoid or minimize them as much as possible. There are ways to lessen these side effects. For example, drugs can be given before or along with the chemotherapy to prevent or reduce nausea and vomiting.
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. With careful monitoring, such side effects are rare. If serious side effects occur, the chemotherapy may have to be reduced or stopped, at least temporarily. Careful monitoring and adjustment of drug doses is important because some side effects to organs can be permanent.
Monoclonal Antibodies
Monoclonal antibodies are special immune proteins made in the lab. They are directed toward specific molecules on the surface of cells. One particular monoclonal antibody called rituximab (Rituxan) has been effective in treating many types of lymphomas and has also helped a few patients with Castleman disease. Rituximab is given by IV injection, usually once a week for 4 weeks. Some people may have an allergic reaction with fever and need treatment with drugs such as acetaminophen (Tylenol) and diphenhydramine (Benadryl).
Antiviral Drugs
Because Castleman disease is associated with the HHV-8, doctors have had some success in treating a few patients with multicentric Castleman with drugs that kill this virus. One drug that was used is called ganciclovir.