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 (PET) scan may help evaluate Castleman disease and monitor treatment response.
- Lymph node biopsy. This test is essential to diagnose Castleman disease and rule out related diseases, 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. But the condition 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.
You'll likely need follow-up exams, including imaging and laboratory tests, to check that the disease hasn't come back.
Treatment of oligocentric Castleman disease, a rare subtype, is usually similar to that of UCD. But more research is needed.
HHV-8-positive multicentric Castleman disease
Rituximab (Rituxan, Ribani, others) 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 issues.
When siltuximab doesn't work, other treatments such as rituximab (Rituxan, Ribani, others) and sirolimus (Rapamune) may be used.
Preparing for your appointment
You may be referred to a doctor trained in treating blood conditions. This type of doctor is called a hematologist.
What you can do
- Write down the symptoms you have had and how long you've had them.
- 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 need 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 these questions 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 ongoing or do they come and go?
- How bad are your symptoms?
- Does anything seem to improve your symptoms?
- What, if anything, seems to worsen your symptoms?
- Do you have any other health conditions, such as lymphoma, HIV/AIDS or Kaposi sarcoma?
Sept. 18, 2026