Castleman Disease

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    William C. Shiel Jr., MD, FACP, FACR

    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

Castleman disease definition and facts

  • Castleman disease is a rare condition affecting lymph nodes, and actually is a group of related conditions.
  • Castleman disease is not cancer, although some of the methods used to treat Castleman disease also are used to treat cancers.
  • There are two main forms of Castleman disease, unicentric (localized) and multicentric (found in different sites throughout the body).
  • Signs and symptoms of Castleman disease occur most often with the multicentric form and can include
  • The diagnosis of Castleman disease involves examination of a tissue sample (biopsy) by a pathologist.
  • Treatment for Castleman disease is complex, and can involve a number of different types and classes of medications such as chemotherapy drugs, immunotherapy drugs, immunomodulator drugs, anti-viral drugs, and corticosteroids.
  • Surgery may be used to treat unicentric Castleman disease or to help relieve symptoms in the multicentric form.
  • Radiation therapy is another form of treatment sometimes used to destroy the abnormal tissue in Castleman disease.
  • Giant lymph node hyperplasia and angiofollicular lymph node hyperplasia (AFH) are other names for Castleman disease.

What is Castleman disease?

Castleman disease is a rare disease of lymph nodes and similar tissues. Sometimes referred to as Castleman's disease, it is actually a group of so-called lymphoproliferative disorders, meaning disorders that are accompanied by abnormal growth (or proliferation) of the lymphatic tissue. The lymphatic (or lymphoid) tissue consists of different types of cells that are involved in the immune response, predominantly white blood cells known as lymphoctyes. Lymphatic tissue is found in lymph nodes, the tonsils, thymus, spleen, bone marrow, and gastrointestinal (GI) tract.

Abnormal growth of lymphoid tissue also is characteristic of lymphoma, a type of cancer, but Castleman disease is not cancer. Other names for Castleman disease are giant lymph node hyperplasia and angiofollicular lymph node hyperplasia (AFH).

Even though Castleman disease is not considered to be cancer, one type of this disease (multicentric Castleman disease, see below) behaves very much like lymph node cancer (lymphoma), and can lead to serious health risks.

Medically Reviewed by a Doctor on 10/5/2016

Subscribe to MedicineNet's Cancer Report Newsletter

By clicking Submit, I agree to the MedicineNet's Terms & Conditions & Privacy Policy and understand that I may opt out of MedicineNet's subscriptions at any time.

Health Solutions From Our Sponsors