Diagnosis

After reviewing your medical history and doing a detailed physical exam, your healthcare professional may recommend:

Blood and urine tests. These help rule out other infections or diseases. These tests also can find anemia and changes in blood proteins that can be typical of Castleman disease.
Imaging tests. These tests can find enlarged lymph nodes or an enlarged liver or spleen. A CT scan of the neck, chest, abdomen and pelvis may be used. A positron emission tomography scan, also known as a PET scan, may be used to diagnose Castleman disease. PET scans also can show whether a treatment is working.
Lymph node biopsy. This test is essential to diagnose Castleman disease and rule out related disorders, such as lymphoma. In a biopsy, a tissue sample from an enlarged lymph node is removed and looked at in a laboratory.

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Treatment

Treatment depends on the type of Castleman disease you have.

Unicentric Castleman disease

Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). If the lymph node is in the chest or abdomen, major surgery may be needed.

Surgery to remove the enlarged lymph node usually cures UCD. However, UCD sometimes comes back. If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. If medicines don't work, radiation therapy may be an option.

More research into the treatment of oligocentric Castleman disease is needed, but treatment is usually similar to that of UCD.

You'll likely need follow-up exams, including imaging and laboratory tests, to check that the disease hasn't come back.

HHV-8-positive multicentric Castleman disease

Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. Rituximab is highly effective, but sometimes medicines called antivirals and chemotherapies are needed. Antiviral medicines can block the activity of HHV-8 or HIV, and chemotherapies can get rid of extra immune cells.

Idiopathic multicentric Castleman disease

Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. In the U.S., siltuximab is the only medicine approved by the Food and Drug Administration (FDA) for the treatment of idiopathic MCD. People who get better after taking siltuximab tend to have successful long-term treatment. This drug blocks the action of a protein called interleukin-6. The bodies of people who have idiopathic MCD produce too much of this protein.

People who are critically ill with idiopathic MCD often receive treatment with medicines called corticosteroids. They also may need chemotherapy. Corticosteroids such as prednisone can help control inflammation. Chemotherapy can get rid of immune cells that are causing problems.

When siltuximab doesn't work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.

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Preparing for your appointment

You may be referred to a doctor trained in treating blood disorders. This type of doctor is 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 conditions.
Make a list of all medicines, vitamins and supplements that you're taking, including the doses.

Questions to ask your doctor

What's the most likely cause of my 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 healthcare professional, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your healthcare 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:

When did you first have symptoms?
Have your symptoms been continuous or occasional?
How bad are your symptoms?
Does anything seem to improve your symptoms?
What, if anything, appears to worsen your symptoms?
Do you have any other health conditions, such as lymphoma, HIV/AIDS or Kaposi sarcoma?

By Mayo Clinic Staff

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Symptoms & causes Doctors & departments

Aug. 06, 2024

Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/12656/castleman-disease. Accessed Jan. 11, 2023.
Fajgenbaum DC. Unicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed Jan. 11, 2023.
Fajgenbaum DC. HHV-8-associated multicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed Jan. 11, 2023.
Haap M, et al. Clinical, laboratory and imaging findings in Castleman's disease: The subtype decides. Blood Reviews. 2018; doi:10.1016/j.blre.2017.11.005.
Van Rhee F, et al. Treatment of idiopathic Castleman disease. Hematology/Oncology Clinics of North America. 2018; doi:10.1016/j.hoc.2017.09.008.
Multicentric Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease. Accessed Jan. 11, 2023.
Fajgenbaum DC. HHV-8-negative/idiopathic multicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed Jan. 13, 2023.
Dispenzieri A, et. al. Overview of Castleman disease. Blood. 2020; doi:10.1182/blood.2019000931.
Van Rhee F, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood advances. 2020; doi:10.1182/bloodadvances.202000334.

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