After reviewing your medical history and doing a detailed physical exam, your health care provider may recommend:

  • Blood and urine tests, to help rule out other infections or diseases. These tests also can reveal anemia and abnormalities in blood proteins that are sometimes typical of Castleman disease.
  • Imaging tests, to look for enlarged lymph nodes, liver or spleen. A CT scan of your neck, chest, abdomen and pelvis may be used. Positron emission tomography, also known as PET scans, may be used to diagnose Castleman disease and to assess whether a treatment is effective.
  • Lymph node biopsy, to distinguish 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.

More Information


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 needed.

If surgical removal isn't possible, medicine 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 medicines 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 made in excess in people who have multicentric Castleman disease.
  • Chemotherapy. This type of medicine can slow the overgrowth of lymphatic cells. Your provider 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.

Preparing for your appointment

You may be referred to a provider who is trained in treating blood disorders called a hematologist.

What you can do

  • Write down the symptoms you have been experiencing and for how long.
  • Write down key medical information, including other health problems.
  • Make a list of all medicines, vitamins and supplements that you're taking.

Questions to ask your doctor:

  • What's the most likely cause of my signs and symptoms?
  • What kinds of tests do I need? Do they require any special preparation?
  • What treatment do you recommend? Do I need surgery?

In addition to the questions that you've prepared to ask your health care provider, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your health care team is likely to ask you a number of questions. Being ready to answer them may leave time to go over points you want to spend more time on. You may be asked:

  • Do you have any other health conditions, such as HIV/AIDS or Kaposi's sarcoma?
  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • Does anything seem to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
May 03, 2024
  1. Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/12656/castleman-disease. Accessed Jan. 11, 2023.
  2. Fajgenbaum DC. Unicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed Jan. 11, 2023.
  3. Fajgenbaum DC. HHV-8-associated multicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed Jan. 11, 2023.
  4. Haap M, et al. Clinical, laboratory and imaging findings in Castleman's disease: The subtype decides. Blood Reviews. 2018;32:225.
  5. Van Rhee F, et al. Treatment of idiopathic Castleman disease. Hematology/Oncology Clinics of North America. 2018;32:89.
  6. Multicentric Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease. Accessed Jan. 11, 2023.
  7. Fajgenbaum DC. HHV-8-negative/idiopathic multicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed Jan. 13, 2023.
  8. Dispenzieri, A, et. al. Overview of Castleman disease. Blood. 2020;135:16.


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