People with unicentric Castleman disease often notice no signs or symptoms. The diseased lymph node may be found during screening or treatment for another illness.

If unicentric or multicentric Castleman disease is suspected, your doctor is likely to start with a thorough physical examination of your lymph nodes, to determine their size and consistency.

Your doctor may then 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. 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. Depending on the location of the lymph node, the biopsy may be done under local anesthesia or during more extensive surgery.


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

Surgery usually isn't an option for multicentric Castleman disease because of the number of lymph nodes involved. However, surgery to remove an enlarged spleen may be an option to help ease symptoms.

Treatment 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.

The options include:

  • Monoclonoal antibodies, to block the action of the IL-6 protein that contributes to cell overgrowth. Your doctor may recommend initial treatment with a monoclonal antibody, such as siltuximab (Sylvant), if you don't have organ damage or HIV or HHV-8 infection.
  • Chemotherapy, to slow overgrowth of lymphatic cells. Your doctor may recommend adding chemotherapy if the disease doesn't respond to monoclonal antibodies or if you have organ failure.
  • Corticosteroids, to control inflammation.
  • Antiviral drugs, to block the activity of HHV-8 or HIV if you have one or both of those viruses.
  • Thalidomide (Thalomid), to block the action of the IL-6 protein. Thalidomide is an immune-system modulator that has been shown to be effective at inducing remission in Castleman disease.

Coping and support

A diagnosis of Castleman disease can be challenging because the condition is rare and often occurs with other serious illnesses, such as HIV/AIDS and Kaposi's sarcoma. To help you cope with Castleman disease:

  • Find someone to talk with. You may feel comfortable discussing your feelings with a friend or family member, or you might prefer meeting with a formal support group.
  • Set reasonable goals. Having goals helps you feel in control and can give you a sense of purpose. But choose goals that you can reach.

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. 27, 2014
  1. AskMayoExpert. What are the symptoms of Castleman disease? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
  2. Brown JR, et al. Unicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
  3. Aster JC, et al. Multicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
  4. Dispenzieri A, et al. The clinical spectrum of Castleman's disease. American Journal of Hematology. 2012;87:997.
  5. Fajgenbaum DC, et al. HHV-8-negative, idiopathic multicentric Castleman disease: Novel insights into biology, pathogenesis, and therapy. Blood. 2014;123:2924.
  6. Robinson D, et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: Results from two US treatment centers. British Journal of Haematology. 2014;165:39.
  7. Talat N, et al. Surgery in Castleman's disease: A systematic review of 404 published cases. Annals of Surgery. 2012;255:677.