Castleman disease

Castleman disease

overview

Castleman disease is a rare condition that causes an overgrowth of cells in your body's lymph nodes. The most common form of the disease 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 herpesvirus type 8 (HHV-8) and human immunodeficiency virus (HIV).

Treatment and outlook vary depending on the type 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 do not notice any signs or symptoms. The enlarged lymph node may be detected during a physical exam or an imaging test for an unrelated problem.

Some people with unicentric Castleman disease may experience signs and symptoms that are more common in multicentric Castleman disease, including:

• Fieber
• Unbeabsichtigter Gewichtsverlust
• Ermüdung
• Nachtschweiß
• Brechreiz
• Vergrößerte Leber oder Milz

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

When to go to the doctor?

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

Causes

It is 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 almost all HIV-positive people with Castleman disease and in about half of HIV-negative people with Castleman disease.

Risk factors

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

The risk of developing multicentric Castleman disease is higher in people 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 can lead to life-threatening infections or organ failure. People who also have HIV/AIDS generally have the worst outcomes.

Both types of Castleman disease can increase your risk of lymphoma.

Sources:

1. Castleman-Krankheit. Informationszentrum für genetische und seltene Krankheiten. https://rarediseases.info.nih.gov/diseases/12656/castleman-disease. Abgerufen am 14. Juni 2018.
2. Fajgenbaum DC. Unizentrische Castleman-Krankheit. https://www.uptodate.com/contents/search. Abgerufen am 14. Juni 2018.
3. Fragen Sie MayoExpert. Castleman-Krankheit (Erwachsener). Rochester, Minnesota: Mayo Foundation for Medical Education and Research; 2017.
4. Haap M. et al. Klinische, Labor- und bildgebende Befunde bei Morbus Castleman: Der Subtyp entscheidet. Blut Bewertungen. 2018;32:225.
5. Van Rhee F, et al. Behandlung der idiopathischen Castleman-Krankheit. Hämatologie/Onkologie-Kliniken Nordamerikas. 2018;32:89.
6. Multizentrische Castleman-Krankheit. Informationszentrum für genetische und seltene Krankheiten. https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease. Abgerufen am 15. Juni 2018.
7. Fajgenbaum DC. HHV-8-assoziierte multizentrische Castleman-Krankheit. https://www.uptodate.com/contents/search. Abgerufen am 14. Juni 2018.