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Castleman disease (CD) is a disease of the lymph nodes -- groups of bean-sized glands throughout your body that help you fight infections. It makes the cells in and around those nodes grow too much and too fast.

But even though Castleman disease involves an overgrowth of cells, it is not cancer. That’s because the cell growth has a different cause -- faulty signals between cells rather than a defect in your genes.

The condition is rare -- about one in 6,500 Americans has it.  People of all ages can get it, but it’s more common in adults. Infection by certain viruses, like HIV, seems to cause at least some cases of Castleman disease, but about half of cases have no clear cause.

The disease is also called giant lymph node hyperplasia and angiofollicular lymph node hyperplasia (AFH). It’s named for the doctor who first described it in the 1950s, Benjamin Castleman. 

Types of Castleman Disease

There are two main types of CD: localized, or unicentric, and multicentric. They affect people in different ways.

Localized Castleman disease is more common among 20- to 30-year-olds. It affects a single lymph node or a single region of lymph nodes, usually in the chest or belly. It makes them grow larger than normal.

The symptoms you have with localized CD depend on which of your lymph nodes are affected:

  • If it’s in your neck, groin or underarm, you may first notice a lump under your skin.
  • In your chest, oversized lymph nodes can press against your windpipe or airways into your lungs, which can make it hard to breathe.
  • In your belly, they might cause pain, make you feel full, or make it hard to eat.

And some people with localized CD have no symptoms at all.

Doctors can usually cure localized Castleman disease by removing the affected lymph nodes with surgery.

Multicentric Castleman disease is the more serious and more complex type. It affects more than one group of lymph nodes and can also affect other parts of the lymph system. It’s most common in 40- to 60-year-olds. 

There are two types of MCD. One is caused by a virus called human herpes virus-8. You might be more likely to get HHV-8 if you have HIV or take medications that turn down your immune system.

Doctors don’t know what causes the other type of MCD, but they call it iMCD or HHV-8-negative MCD, since it’s not related to HHV-8. 

Symptoms for MCD can include

  • fever and night sweats
  • nausea and vomiting
  • loss of appetite
  • weight loss
  • a buildup of fluid in your belly
  • bruising and easy bleeding
  • cherry-like spots on your skin
  • numbness in your hands and feet

Anemia -- having too few red blood cells -- is very common if you have MCD. It can give you shortness of breath and fatigue.

You might also have a buildup of abnormal proteins in your body.  This can damage your heart, kidneys, and nerves, and also cause diarrhea or other digestive problems.

Because MCD makes your immune system weaker, you’re more likely to get a serious infection.

And even though multicentric Castleman disease isn’t cancer, it can raise your odds of getting lymphoma, a cancer of the lymph system.

Since MCD affects many areas of your body at once, surgery usually isn’t a good treatment choice. But there are other ways to treat the disease and control your symptoms.

WebMD Medical Reference

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