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.

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 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.

Preparing for your appointment

You may be referred to a doctor who specializes in treating blood disorders (hematologist).

What you can do

  • Write down the symptoms you have been experiencing and for how long.
  • Write down key medical information, including other conditions.
  • Make a list of all medications, 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 doctor, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may make 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?
Aug. 01, 2020
  1. Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/12656/castleman-disease. Accessed June 14, 2018.
  2. Fajgenbaum DC. Unicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed June 14, 2018.
  3. AskMayoExpert. Castleman disease (adult). Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2017.
  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 June 15, 2018.
  7. Fajgenbaum DC. HHV-8-associated multicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed June 14, 2018.


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