What Is Castleman Disease?

Castleman disease (CD) is a rare disease of lymph nodes and related tissues.It was first described by Dr. Benjamin Castleman in the 1950s. It is also known as Castleman’s disease, giant lymph node hyperplasia, and angiofollicular lymph node hyperplasia (AFH). CD is not cancer. Instead, it is 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 (cancers of lymph nodes).

Even though CD is not officially a cancer, one form of this disease (known as multicentric Castleman disease) acts very much like lymphoma. In fact, many people with this disease eventually develop lymphomas. And like lymphoma, CD is often treated with chemotherapy or radiation therapy. This is why it is included in the American Cancer Society’s cancer information. (For information about lymphoma, see Hodgkin Disease and Non-Hodgkin Lymphoma.).

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

Doctors can group CD in a number of ways, and they are still trying to determine which classification (or combination of them) provides the most helpful information.

CD is classified by on how much of the body it affects. The main forms of CD are called localized and multicentric. They affect people very differently.

Localized (unicentric) Castleman disease

This is the more common type of CD. 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 grow.

Enlarged lymph nodes in the chest can press on the windpipe (trachea) or smaller breathing tubes going into the lungs (bronchi), causing breathing problems. If the enlarged nodes are in the abdomen, the person might have pain, a feeling of fullness, or trouble eating. 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 Castleman disease (MCD) affects more than one 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. You can read more about HIV infection in HIV, AIDS, and Cancer.

People with MCD 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).

MCD can weaken the body’s immune system, making it hard to fight infection. Infections in people with MCD can be very serious, even life threatening. MCD also increases the risk of developing lymphoma, a cancer of lymphoid tissue, which can often be hard to treat.

Microscopic subtypes of CD

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

  • The hyaline vascular type is most common. It tends to be localized, in which case people often have few symptoms and usually have a good outlook, but in rare cases it can be multicentric.
  • The plasma cell type is more likely to cause symptoms and to be multicentric, but it is sometimes localized.
  • The mixed subtype shows areas of both hyaline vascular and plasma cell types. It occurs less often.
  • The plasmablastic type was recognized more recently. Like the plasma cell type, it is usually multicentric, usually causes symptoms, and has a less favorable outlook.

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

Subtypes of CD based on viral infections

Infection with certain viruses plays a role in at least some cases of CD.

Multicentric CD is more common in people infected with HIV, the virus that causes AIDS. Doctors sometimes group patients with multicentric CD into those who are infected with HIV (HIV positive) and those who are not infected (HIV negative).

In recent years, it’s become clear that another virus, known as human herpesvirus-8 (HHV-8) or Kaposi sarcoma herpesvirus (KSHV), is often found in the lymph node cells of people with multicentric CD. In fact, HHV-8 is found in the lymph nodes of nearly all CD patients who are HIV positive. Some doctors have suggested classifying CD based on whether the cells contain HHV-8.

The American Cancer Society medical and editorial content team

Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.

Castleman Disease Collaborative Network. About Castleman disease.  http://www.cdcn.org/about-castleman-disease/. Accessed January
5, 2018.  

Gross TG. Immunodeficiency and cancer. In: Neiderhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 5th ed. Philadelphia, PA. Elsevier; 2014:1991-2017. 

Munshi NC, Jaggannath S. Plasma cell neoplasms. In: Hematology: Basic Principles and Practice, 7th ed. Hoffman R, Benz Jr. E, Silberstein
LE, Heslop H, Weitz JI, Anastasi J, Salama ME, Abutalib SA, eds. Philadelphia, PA. Elsevier; 2018: 1381-1418. 

National Organization for Rare Disorders. Castleman disease. Accessed at https://rarediseases.org/rare-diseases/castlemans-disease/ on January 5, 2018. 

Oksenhendler E, Boutboul D, Fajgenbaum D, Mirouse A, Fieschi C, Malphettes M, Vercellino L, Meignin V, Gerard L, Galicier L. The full spectrum of Castleman disease: 273 patients studied over 20 years. Hematol Oncol Clin North Am. 2018;32(1):75-88.  

Rajkumar SV, Dispenzieri A. Multiple myeloma and related disorders In: Neiderhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 5th ed. Philadelphia, PA. Elsevier; 2014:1991-2017. 

Wong RSM. Unicentric Castleman disease. Hematol Oncol Clin North Am. 2018;32(1):65-73. 

Yarchoan R, Uldrick TS, Polizzotto MN, Little RF. HIV-associated malignancies. In: DeVita VT, Lawrence TS, Rosenberg SA, eds. DeVita, Hellman,
and Rosenberg’s Cancer: Principles and Practice of Oncology
. 10th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2015:1780-1792. 

Last Medical Review: January 20, 2017 Last Revised: February 1, 2018

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