Overview

Castleman disease is a rare disorder that involves an overgrowth of cells in your body's lymph nodes. The most common form of the disorder affects a single lymph node (unicentric Castleman disease), usually in the chest or abdomen.

Multicentric Castleman disease affects multiple lymph nodes throughout the body and has been associated with human herpes virus type 8 (HHV-8) and human immunodeficiency virus (HIV).

Treatment and outlook vary, depending on the variety of Castleman disease you have. The type that affects only one lymph node can usually be successfully treated with surgery.

Symptoms

Many people with unicentric Castleman disease don't notice any signs or symptoms. The enlarged lymph node may be detected during a physical exam or an imaging test for some unrelated problem.

Some people with unicentric Castleman disease might experience signs and symptoms more common to multicentric Castleman disease, which may include:

  • Fever
  • Unintended weight loss
  • Fatigue
  • Night sweats
  • Nausea
  • Enlarged liver or spleen

The enlarged lymph nodes associated with multicentric Castleman disease are most commonly located in the neck, collarbone, underarm and groin areas.

When to see a doctor

If you notice an enlarged lymph node on the side of your neck or in your underarm, collarbone or groin area, talk to your doctor. Also call your doctor if you experience a persistent feeling of fullness in your chest or abdomen, fever, fatigue, or unexplained weight loss.

Causes

It's not clear what causes Castleman disease. However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease.

The HHV-8 virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor that can be a complication of HIV/AIDS. Studies have found that HHV-8 is present in nearly all HIV-positive people who have Castleman disease, and in about half of HIV-negative people with Castleman disease.

Risk factors

Castleman disease can affect people of any age. But the average age of people diagnosed with unicentric Castleman disease is 35. Most people with the multicentric form are in their 50s and 60s. The multicentric form is also slightly more common in men than in women.

The risk of developing multicentric Castleman disease is higher in people who are infected with a virus called human herpesvirus 8 (HHV-8).

Complications

People with unicentric Castleman disease usually do well once the affected lymph node is removed. Multicentric Castleman disease may lead to life-threatening infections or organ failure. People who also have HIV/AIDS generally have the worst outcomes.

Having either variety of Castleman disease may increase your risk of lymphoma.

Aug. 30, 2018
References
  1. Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/12656/castleman-disease. Accessed June 14, 2018.
  2. Fajgenbaum DC. Unicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed June 14, 2018.
  3. AskMayoExpert. Castleman disease (adult). Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2017.
  4. Haap M, et al. Clinical, laboratory and imaging findings in Castleman's disease: The subtype decides. Blood Reviews. 2018;32:225.
  5. Van Rhee F, et al. Treatment of idiopathic Castleman disease. Hematology/Oncology Clinics of North America. 2018;32:89.
  6. Multicentric Castleman disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease. Accessed June 15, 2018.
  7. Fajgenbaum DC. HHV-8-associated multicentric Castleman disease. https://www.uptodate.com/contents/search. Accessed June 14, 2018.