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Castleman disease

Published March 7, 2018
• Reviewed by a UI Health Care Provider
on March 1, 2018

What is Castleman disease (CD)?

CD is a rare, non-cancerous disease of the lymphatic system. It often presents no symptoms, but it can develop into lymphoma.

There are two forms of CD: unicentric and multicentric. Unicentric CD affects a single lymph node region, while multicentric CD affects multiple lymph node regions and lymphatic tissues.

Risk factors

CD is a rare condition, and not much is known about its risk factors. HIV infection can increase the risk of CD, as can the development of AIDS.

Signs and symptoms

Not everyone with CD experiences symptoms. The most common symptoms result from the swelling of the lymph nodes. In unicentric CD, swelling occurs in a single region leading to symptoms such as:

  • Chest or abdominal pressure
  • Swelling or lumps under the skin
  • Excessive fatigue
  • Dramatic weight loss
  • Cough
  • Anemia

Multicentric CD can lead to chronic swelling throughout the body, which may lead to a variety of symptoms such as:

  • General weakness
  • Fatigue
  • Night sweats and recurrent fevers
  • Shortness of breath or difficulty breathing
  • Nausea, vomiting, and loss of appetite
  • Dramatic weight loss
  • Swelling or lumps under the skin in multiple areas

Diagnosis

CD diagnosis begins with the discovery of swollen lymph nodes. Often this swelling is the result of an infection in the area.

The exploration of the patient’s medical history, blood tests, and imaging studies may be performed to see if the symptoms are the result of non-CD conditions.

A final diagnosis of CD may only be made by a biopsy of an enlarged lymph node.

Treatment

Treatment options for CD vary depending on whether the condition is unicentric or multicentric. In unicentric CD, the swollen tissue is surgically removed. Additional therapy may be used prior to surgery, such as the use of steroids to shrink the mass.

A majority of unicentric CD conditions are treated completely by surgical removal of the tumor. In general, unicentric CD rarely progresses to lymphoma.

As multicentric CD involves multiple lymph node areas, surgery typically is not an option. Instead, other therapies such as medication may be used to minimize cell growth and provide symptom relief.