Castelman`s disease

After a thorough physical exam, your doctor may recommend:

Blood and urine tests, to help rule out other infections or diseases. These tests can also reveal anemia and abnormalities in blood proteins that are sometimes characteristic of Castleman disease.

Imaging tests, to detect enlarged lymph nodes, liver or spleen. A CT scan or MRI of your neck, chest, abdomen and pelvis may be used. Positron emission tomography (PET) scans also may be used to diagnose Castleman disease and to assess whether a treatment is effective.

Lymph node biopsy, to differentiate Castleman disease from other types of lymphatic tissue disorders, such as lymphoma. A tissue sample from an enlarged lymph node is removed and examined in the laboratory.

Treatment depends on the type of Castleman disease you have.

Unicentric Castleman disease

Unicentric Castleman disease can be cured by surgically removing the diseased lymph node. If the lymph node is in your chest or abdomen — which is often the case — major surgery may be required.

If surgical removal isn't possible, medication may be used to shrink the lymph node. Radiation therapy also may be an effective way to destroy the affected tissue.

You'll need follow-up exams, including imaging, to check for relapse.

Multicentric Castleman disease

Treatment for multicentric Castleman disease generally involves medications and other therapies to control cell overgrowth. Specific treatment depends on the extent of your disease and on whether you have HIV or HHV-8 infection or both.

Treatment options for multicentric Castleman disease may include:

Immunotherapy. The use of drugs such as siltuximab (Sylvant) or rituximab (Rituxan) can block the action of a protein that is produced in excess in people who have multicentric Castleman disease.

Chemotherapy. This type of medication can slow the overgrowth of lymphatic cells. Your doctor may recommend adding chemotherapy if the disease doesn't respond to immunotherapy or if you have organ failure.

Corticosteroids. Drugs such as prednisone can help control inflammation.

Antiviral drugs. These drugs can block the activity of HHV-8 or HIV if you have one or both of those viruses.