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, usually in the chest or abdomen. This form is called unicentric Castleman disease.
Multicentric Castleman disease, or MCD, affects multiple lymph nodes throughout the body. There are 3 types:
- HHV-8-associated MCD. This type is associated with human herpes virus type 8, called HHV-8, and human immunodeficiency virus, or HIV.
- Idiopathic MCD. The cause of this type is unknown. This is also called HHV-8-negative MCD.
- POEMS-associated MCD. This type is associated with another condition called POEMS syndrome. POEMS syndrome is a rare blood disorder that damages your nerves and affects other parts of your body.
Treatment and outlook vary, depending on the type of Castleman disease you have. The type that affects only one lymph node, unicentric Castleman disease, 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 found during a physical exam or an imaging test for some unrelated problem.
Some people with unicentric Castleman disease might have signs and symptoms that are more commonly seen in multicentric Castleman disease, which may include:
- Fever.
- Unintended weight loss.
- Fatigue.
- Night sweats.
- Enlarged liver or spleen.
The enlarged lymph nodes associated with multicentric Castleman disease are most commonly found 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 health care provider. Also call your care team if you have 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 herpes virus 8, or HHV-8, is associated with the HHV-8 version of multicentric Castleman disease.
Studies have found that HHV-8 is present in nearly all HIV-positive people who have Castleman disease and in fewer than of 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 HIV.
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.
Jan. 24, 2023