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Cancer Medicine :: Castleman disease

Castleman disease

General treatment

Once Castleman disease (CD) has been diagnosed, your health care team will discuss treatment options with you. Several different types of treatment can be used for CD. The treatment options depend on the whether the CD is unicentric or multicentric, as well as other factors. Of course, no two patients are exactly alike, and treatment is often tailored to each patient's situation.

Based on your treatment options, you may have different types of doctors on your treatment team. These doctors may include:

A surgeon

A hematologist: a doctor who treats disorders of the blood and lymph system, including CD.

A medical oncologist: a doctor who treats cancer and similar diseases with medicines.

A radiation oncologist: a doctor who treats cancer and similar diseases with radiation therapy.

Many other specialists may be involved in your care as well, including nurse practitioners, nurses, nutrition specialists, social workers, and other health professionals.

It is important to discuss all of your treatment options as well as their possible side effects with your doctors to help make the decision that best fits your needs. In choosing a treatment plan, consider your health and the type of CD. Be sure that you understand all the risks and side effects of the various treatments before making a decision.

CD is a rare disease, so not many doctors have much experience in treating it. If time permits, it is often a good idea to seek a second opinion. Getting a second opinion can give you more information and help you feel confident about the treatment plan that you choose. Your doctor should be willing to help you find another cancer doctor who can give you a second opinion.

The next few sections describe the types of treatment used for Castleman disease. This is followed by a discussion of the typical treatment options based on the type of CD, as well as other factors when these are important.

Surgery for Castleman disease

Surgery is often used to obtain a tissue sample to diagnose Castleman disease (CD). A lymph node biopsy is usually a minor procedure, and patients can often go home afterwards.

Surgery also works well to treat localized (unicentric) CD. The type of surgery depends on where the disease is located.

If the involved lymph node or nodes are in a place that is easy to get to, such as in the armpit, then surgery is usually straightforward. In many cases the person may even be able to go home the same day after the surgery.

When the enlarged lymph nodes are in a place that is hard to get to, like deep in the chest or abdomen, surgery is more complex. The patient often has some pain and may need to stay in the hospital for a few days after the operation.

Aside from pain, possible side effects of surgery can include poor wound healing, bleeding at the surgery site, and infection. Depending on the site of surgery, other side effects are also possible.

Radiation therapy for Castleman disease

Radiation therapy uses high-energy radiation to kill cells. Radiation focused from a source outside the body is calledexternal beam radiation. Radiation therapy has sometimes been used instead of surgery to treat localized Castleman disease (CD). Some doctors may also use it as part of the treatment for multicentric CD.

The treatment is much like getting an x-ray, but the radiation is more intense. The procedure itself is painless. Before the treatments start, the radiation team takes careful measurements to determine the correct angles for aiming the radiation beams and the proper dose. Each treatment lasts only a few minutes, although the setup time – getting you into place for treatment – usually takes longer. Most often, radiation treatments are given 5 days a week for several weeks.

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. Radiation to the chest area may damage the heart and lungs. This might eventually lead to problems such as shortness of breath or an increased risk of heart attacks. Radiation may also make the side effects of chemotherapy worse if they both are given at the same time.

Corticosteroids for Castleman disease

Corticosteroids are a group of drugs related to hormones made in the body by the adrenal glands. These drugs inhibit the immune system, so they 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 (CD) are helped by treatment with these drugs.

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

Side effects of corticosteroids can include increased blood sugar (which may lead to diabetes), depression, increased risk of 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 for Castleman disease

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 Castleman disease (CD). Chemo may be used alone, in combination with corticosteroids, or in combination with radiation therapy (called chemoradiation).

Many chemo drugs have been used to treat patients with multicentric CD. The drugs 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. But because CD is so rare, there is not a lot of information on which chemo treatment is best or even how well it works.

Doctors give chemo in cycles, in which a period of treatment is followed by a rest period to allow the body time to recover. Each chemo cycle generally lasts for several weeks. Most chemo treatments are given on an outpatient basis (in the doctor's office or clinic or hospital outpatient department) but some may require a hospital stay. Sometimes a patient takes one drug combination for several cycles and then later is switched to a different one.

Chemo drugs attack cells that are dividing quickly, which is why they work against cancer and diseases like CD. But other cells in the body, such as those in the bone marrow, the lining of the mouth and intestines, and the hair follicles, also divide quickly. These cells are also likely to be affected by chemotherapy, which can lead to certain side effects.

The side effects of chemo depend on the type and dose of drugs given and the length of time they are taken. These side effects can include: Hair loss,Mouth sores,Loss of appetite, Nausea and vomiting, Diarrhea, Increased risk of 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 help prevent or reduce nausea and vomiting. Most side effects are temporary and go away after treatment is finished.

Infections can be very serious in people getting chemo. A low white blood cell count is an important risk factor for serious infections, so 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.

If your white blood cell counts are very low during treatment, you can help reduce your risk of infection by carefully limiting your exposure to germs. During this time, your doctor may advise you to:

Wash your hands often.

Avoid fresh, uncooked fruits and vegetables and other foods that might carry germs.

Avoid fresh flowers and plants because they may carry mold.

Make sure other people wash their hands before they touch you.

Avoid large crowds and people who are sick (wearing a surgical mask offers some protection in these situations).

Certain chemo drugs can have other, more specific side effects. Organs that could be damaged by certain chemo drugs include the kidneys, liver, testicles, ovaries, brain, heart, and lungs. Many of the drugs used to treat 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 for a short time. Your doctor will carefully monitor and adjust drug doses because some side effects can be permanent.

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.

Thalidomide: 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: 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.

Antiviral drugs for Castleman disease

Multicentric Castleman disease (CD) is sometimes 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, such as ganciclovir.

Many patients with HIV infection are treated with anti-retroviral therapy to keep the HIV in check. The effect of therapy for HIV on CD is not clear.

After Treatment

For many people with Castleman disease (CD), treatment may remove or destroy the disease. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about the CD growing or coming back. (When the disease comes back after treatment, it is called recurrence.) This is a very common concern in people with serious diseases such as CD.

For some people, the CD may never go away completely. These people may get regular treatments with chemotherapy, corticosteroids, or other therapies to help keep the CD in check for as long as possible. Learning to live with CD as a more of a chronic disease can be difficult and very stressful. It has its own type of uncertainty.

Follow-up care

If you have completed treatment, frequent follow-up exams are very important for several years after the treatment is finished. The doctors will continue to watch you for signs of recurrent disease, as well as for short-term and long-term side effects of treatment. It is important that you report any new symptoms to the doctor right away, so that relapse or side effects can be treated.

Checkups usually include careful physical exams, imaging tests such as CT scans when needed, and lab tests to look for signs of CD or treatment side effects.

Almost any type of treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. This is the time for you to talk to your health care team about any changes or problems you notice and any questions or concerns you have.

CD may recur (come back) in some people. Multicentric CD may come back as soon as the first year after treatment. If the CD does recur at some point, further treatment will depend on what treatments you've had before, how long it's been since treatment, and your health.

Some people with multicentric CD (especially those who are HIV-positive) may develop non-Hodgkin lymphoma or Kaposi sarcoma at some point. These cancers can be hard to treat, so it helps to diagnose and treat them as early as possible.

Latest Research

Research into the causes and treatment of Castleman disease (CD) is being done in many medical centers throughout the world. Unfortunately, research into this disease is slowed by the fact that CD is so rare. Still, researchers have begun to study several promising new drugs in recent years for use against CD.

Many patients with multicentric CD have high levels of a protein called interleukin-6 (IL-6). Drugs against IL-6 have been developed and are being tested in CD. One of these drugs, tocilizumab (Actemra®), is a monoclonal antibody that blocks the action of IL-6 by binding to its receptor on lymphocytes. This drug is approved to treat rheumatoid arthritis in the United States, but some doctors may use it to treat CD as well.

Another monoclonal antibody, siltuximab, targets IL-6 itself. This drug has shown very promising results in an early study against CD, but it is only available through clinical trials at this time.

It is unclear how helpful these drugs may be in CD patients who are HIV-positive, since few HIV-positive patients have been in the studies so far. Still, these drugs may offer the best hope for the future treatment of CD.

Other drugs being studied for treatment of CD include:

Sirolimus (Rapamune®): This drug suppresses the immune system by keeping lymphocytes in check. It is often used to help prevent the rejection of organ transplants, but it may also be helpful in CD.

Suramin: This drug is thought to work by stopping IL-6 from attaching to and affecting lymphocytes.

CX-4945: This drug blocks CK2, a protein that helps some cells grow and divide. It is still in the earliest phases of testing.

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