Castleman Disease

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Treating Castleman Disease TOPICS

Treatment of multicentric Castleman disease

Multicentric Castleman disease (CD) is usually much harder to treat than localized CD. Surgery is used to biopsy an affected lymph node to make the diagnosis, but the disease is too widespread to remove it all with surgery. Still, some people are helped when some of the diseased tissue is removed.

There is no standard therapy for multicentric CD. No single treatment works for all patients. Several types of treatment have been shown to help some patients. But because CD is rare, it has been hard for doctors to compare different treatments against each other in clinical trials.

Doctors usually try one or a combination of treatments to try to put the disease in remission. Corticosteroids, chemotherapy, and immunotherapy may be helpful. Radiation is sometimes used. Anti-viral drugs including anti-HIV treatment may also help.

In about half of patients the disease goes away completely with treatment, at least for a time. This is less likely to happen in patients with HIV/AIDS. Even if the HIV infection is under control with drug treatment, the CD is not likely to go away.

Corticosteroids, chemotherapy, and immunotherapy may produce long remissions for some patients. In other patients, the benefit does not last long and the symptoms worsen after the course of therapy is done. Some patients may not be helped by these drugs at all.

In people whose CD is no longer responding to other treatments, some doctors may recommend high-dose chemotherapy followed by a stem cell transplant. There are a few reported cases of this being successful. This is a complex, serious, and often expensive treatment, so it is important to understand what it might entail if you are considering this option. For more on this treatment, see our document, Stem Cell Transplant (Peripheral Blood, Bone Marrow, and Cord Blood Transplants).

The long-term outlook (prognosis) for people with multicentric CD is often not as good as for people with localized CD. Treatment may help, but the disease often comes back within a couple of years. A major concern is that people with multicentric CD are at risk of dying from other causes, like serious infections or progression of the CD to a fast-growing form of lymphoma that is hard to treat. (For more information about lymphoma, please see our document, Non-Hodgkin Lymphoma).

The outlook for multicentric CD tends to be worse if the person also has HIV/AIDS. When someone is HIV-positive, treatment and outlook of CD can be complicated by Kaposi sarcoma and other AIDS-related conditions. These conditions may be less of a problem if the patient is on anti-HIV treatment.

Because multicentric CD can be hard to treat, taking part in a clinical trial of newer treatments may be a good option for some people. (See “What’s new in research and treatment of Castleman disease?” for a description of some newer treatments.)

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