Castleman disease is a rare condition that affects your body’s lymph nodes, causing one or more of them to enlarge. Castleman can become life threatening if not treated, causing infection and organ damage.

Lymph nodes are small glands located throughout your body, in the armpits, neck, groin, and elsewhere.

Your lymph nodes filter a clear fluid called lymph for your body’s lymphatic system. They also store white blood cells, which protect your body against infections by destroying invading bacteria and viruses.

Some people with Castleman disease have no symptoms at all, while other people have symptoms that are similar to the flu. The exact cause of Castleman disease isn’t known, but having HIV is the largest risk factor for developing the condition.

Treatment for Castleman depends on how many lymph nodes are affected, and where. In this article, we’ll overview the types of this disease, possible symptoms, and what treatments are currently available.

Is Castleman disease cancer?

Castleman disease is not cancer. It’s called a “lymphoproliferative disorder.”

However, according to 2015 research, people with Castleman are at an increased risk of developing certain cancers. Among others, these include:

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There are two types of Castleman disease: unicentric and multicentric. Both types affect the lymph nodes, but they vary by the number of lymph nodes involved and the severity of symptoms.

Let’s break down the characteristics of each.


Unicentric Castleman disease (UCD) impacts lymph nodes in only one area of the body. It’s also sometimes called localized Castleman disease. Most people with Castleman disease have this type.


Multicentric Castleman disease (MCD) impacts multiple lymph node regions in the body. MCD is more common in people with HIV or who are immunocompromised. People with MCD are more likely to have noticeable symptoms compared with people with UCD.

The exact cause of Castleman disease is unknown. It’s likely that the different types of Castleman disease have different underlying causes.

Researchers believe that UCD is caused by a genetic mutation, and they are studying multiple different possibilities. A mutation called PDGFRB has been documented in multiple cases of UCD and idiopathic MCD.

Having HIV is a major risk factor in developing MCD. This is due in part to people with HIV having an increased chance of getting a specific virus associated with Castleman.

Almost all cases of MCD are linked to an infection with human herpesvirus type 8 (HHV-8), which can cause the cancer Kaposi sarcoma.

HHV-8 most commonly affects people with HIV or who are immunocompromised. When the disease is not linked to HHV-8, it is called “idiopathic multicentric Castleman disease,” meaning the cause is unknown.

It’s important for people with HIV and Castleman to manage their HIV symptoms for the best possible health outcome and quality of life.

Learn more about HIV treatment.

The symptoms of Castleman disease can vary depending on the type. Often, people with UCD have no symptoms at all.

When UCD does cause symptoms, they can include:

  • unintentional weight loss
  • fever
  • fatigue
  • a swollen lymph node (lump under the skin) in the neck, armpit, collarbone, or groin
  • pressure or a feeling of fullness in the stomach or chest
  • anemia, in some cases, due to a low red blood cell count

MCD causes a wider range of symptoms. People with MCD are a lot more likely to have symptoms that prompt them to visit a healthcare professional.

Symptoms of MCD may include:

Since UCD often causes no symptoms, it’s common for UCD to be discovered during routine lab work or testing for another condition. Your doctor might also notice that your lymph nodes are swollen during a physical exam.

In MCD, the symptoms can be similar to those of many other conditions. Testing will help rule out other health conditions.

Regardless of your Castleman type, the diagnostic process usually begins with a physical exam and blood work.

Your doctor might order imaging tests to look for enlarged lymph nodes throughout your body. These tests may include:

If you have swollen lymph nodes, you might have a tissue biopsy done.

During a biopsy, a healthcare professional will use a hollow needle to remove a sample of tissue. The sample will be evaluated under a microscope to confirm a diagnosis of Castleman disease.

The treatment for Castleman disease depends on:

  • the type of Castleman disease you have
  • which lymph node or nodes are affected
  • whether or not you also have HIV

Treatment plans generally combine several different methods. These include:

  • Surgery. Surgery is often done to remove the affected lymph nodes, but only in UCD. This can prevent or treat UCD’s life threatening complications, mainly organ damage, organ failure, and infection.
  • Radiation treatment. Radiation can be used to shrink tumor (sarcoma) growth. It can be done before surgery to make the removal easier or after surgery to destroy any growth that remains.
  • Immunotherapy. Like radiation, immunotherapy can kill tumor cells. In this case, immunotherapy refers to a special antibody medication (rituximab) that’s given to people with multiple myeloma. This treatment attacks and shrinks the abnormal, overactive immune cells. It can generally be used before or after surgery.

In addition to immunotherapy, treatments for MCD can also include:

  • Chemotherapy. Chemotherapy is used to stop the overgrowth of cancerous cells in the lymphatic system.
  • Corticosteroids. Corticosteroids can help reduce swelling and pain.
  • Antiviral therapy. Antiviral and antiretroviral medications can help get HHV-8 and HIV infections under control. These drugs prevent a virus from multiplying itself, among other effects, making it easier for your immune system to fight it.

Castleman disease is a rare condition that causes swollen lymph nodes. Treatment depends on whether you have unicentric or multicentric Castleman disease.

Some people may experience no symptoms, while others have life threatening complications. A routine physical exam and blood work can diagnose Castleman.

UCD is more common and seems to result from a genetic mutation. MCD almost always results from contracting the HHV-8 virus, and having HIV is a major risk factor.

Treatments for Castleman disease include:

  • immunotherapy
  • surgery
  • radiation
  • chemotherapies

Talk with a doctor if you are experiencing swelling or lumps under the skin anywhere on your body, especially in areas where lymph nodes are located.

Scheduling a yearly physical exam is also a great way to help make sure you do not have any underlying health conditions, even if you have no symptoms.

If you have HIV, consider discussing your risk factors for Castleman disease or other health conditions with a doctor.