Castleman Disease

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What Is Castleman Disease? TOPICS

What is Castleman disease?

Castleman disease (CD) is a disease of lymph nodes and related tissues. It was first described by Dr. Benjamin Castleman in 1956. It was previously called Castleman’s disease. CD is also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia.

CD is not officially a cancer, but the multicentric form of this disease acts very much like lymphoma (cancer of lymph nodes). In fact, many people with this disease eventually develop lymphomas. This is why it is included in the American Cancer Society’s cancer information. (For information about lymphoma, see our documents, Hodgkin Disease and Non-Hodgkin Lymphoma.)

Instead of being called a cancer, CD is often called a lymphoproliferative disorder. This means there is an abnormal overgrowth of cells of the lymph system that is similar in many ways to lymphomas. Like lymphoma, CD is often treated with chemotherapy or radiation therapy.

About lymph nodes and lymphoid tissue

To understand Castleman disease, it helps to know about the body's lymph system.

Lymphoid tissue, also known as lymphatic tissue, is the main part of the immune system. It is formed by different types of cells that work together to help the body fight infections. The main cells in lymphoid tissue are lymphocytes, a type of white blood cell. There are 2 main types of lymphocytes: B cells and T cells.

Lymphoid tissue is found in many places throughout the body, including:

  • Lymph nodes: bean-sized collections of lymphocytes found in small groups throughout the body, including inside the chest, abdomen, and pelvis. They can sometimes be felt under the skin in the neck, under the arms, and in the groin.
  • Thymus: a small organ behind the upper part of the breastbone and in front of the heart. The thymus plays a vital role in development of T cells.
  • Spleen: an organ under the lower part of the rib cage on the left side of the body. The spleen makes lymphocytes and other immune system cells to help fight infection. It also stores healthy blood cells and helps filter the blood.
  • Tonsils and adenoids: collections of lymphoid tissue at the back of the throat. They help protect the body against germs that are breathed in or swallowed.
  • Bone marrow: the soft inner part of certain bones that makes red blood cells, blood platelets, and white blood cells (including lymphocytes).
  • Digestive tract: the stomach, intestines, and other organs, which also have lymphoid tissue.

Types of Castleman disease

The 2 main forms of CD are called localized and multicentric. They affect people very differently.

Localized (Unicentric) Castleman disease

Localized CD only affects a single group of lymph nodes. It is not widespread. Lymph nodes in the chest or abdomen are affected most often. CD causes these lymph nodes to enlarge. These abnormally large lymph nodes may press on other organs and tissues inside the chest or abdomen.

Enlarged lymph nodes in the chest can press on the windpipe (trachea) or smaller breathing tubes (bronchi), causing breathing problems. If the enlarged nodes are in the abdomen, the person might feel pain or pressure in that area. Sometimes the enlarged nodes are in places such as the neck, groin, or underarm area and are first noticed as a lump under the skin.

People with localized CD are usually cured when the affected lymph nodes are removed with surgery.

Multicentric Castleman disease

Multicentric CD affects more than a single group of lymph nodes. It can also affect other organs containing lymphoid tissue. This form sometimes occurs in people infected with human immunodeficiency virus (HIV), the virus that causes AIDS. Multicentric CD is more serious than the localized type, particularly in people with HIV infection.

People with multicentric CD often have problems such as serious infections, fevers, weight loss, fatigue, night sweats, and nerve damage that can cause weakness and numbness. Blood tests often show too few red blood cells (anemia) and high levels of antibodies in the blood (hypergammaglobulinemia).

CD can weaken the immune system severely, making it hard to fight infection. Infections in people with multicentric CD can be very serious and may even lead to death. CD also increases the risk of developing lymphoma, a cancer of lymphoid tissue. This can be fatal.

Microscopic subtypes of Castleman disease

Castleman disease can also be classified based on how the lymph node tissue appears under a microscope. These are called microscopic subtypes.

  • The hyaline vascular type is most common. It tends to be localized, but in rare cases it is multicentric.
  • The plasma cell type is slightly more likely to be multicentric, but it is sometimes localized.
  • The mixed subtype shows areas of both types. It occurs less often.

In choosing treatments, doctors believe that the microscopic type is less important than whether the disease is localized or multicentric.

Last Medical Review: 06/11/2012
Last Revised: 06/11/2012