This rare disease affecting the lymph nodes can increase your risk of developing lymphoma and life threatening infections. But timely treatment can often prevent serious complications.

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Multicentric Castleman disease is an immune system disease that affects the lymph nodes. It’s one of two main types of Castleman disease. The other is unicentric Castleman disease.

Multicentric Castleman disease can cause flu-like symptoms and raise the risk of cancer or serious infections. Treatment depends on the type and severity of the disease, so getting a timely diagnosis is vital to improving your odds of a healthy outcome.

Learn more about Castleman disease.

Multicentric Castleman disease is a rare disease involving lymph nodes in several regions of the body. The other main type of this disease, unicentric Castleman disease, affects one lymph node or one lymph node region. If you have multicentric Castleman disease, the immune system causes multiple lymph nodes to become inflamed for no obvious reason.

Normally, the body’s immune system becomes activated and stays in that mode only when it’s working to stop an infection or disease. This can cause lymph nodes to become swollen and inflamed as they carry white blood cells and other substances the body needs to stop infections. When the threat, such as a bacterial or viral infection, is gone, the immune system—including your lymph nodes—returns to its typical state.

With multicentric Castleman disease, lymph nodes in different parts of the body become inflamed, potentially causing organ damage or a cancer of the lymph system called lymphoma.

Enlarged lymph nodes are one of the main symptoms of multicentric Castleman disease. You may also have an enlarged spleen, an enlarged liver, or both, depending on how far the disease has progressed.

Other common symptoms may include:

A 2023 study suggests that in one-third to one-half of all people with multicentric Castleman disease, the cause is unknown.

Some research also suggests that human herpesvirus (HHV)-8 may lead to multicentric Castleman disease. This is known as HHV-8-associated multicentric Castleman disease.

Doctors diagnose an estimated 4,300 to 5,200 cases of Castleman disease (multicentric or unicentric) in the United States annually. Although the total number of cases is relatively small, the disease can affect anyone at any age.

The only known risk factor for HHV-8-associated multicentric Castleman disease is having HIV. There are no established risk factors for idiopathic multicentric Castleman disease.

Diagnosing multicentric Castleman disease usually starts with a review of symptoms and your medical history. Lab tests and imaging are also necessary to make an accurate diagnosis.

A doctor may order a blood test to check for anemia (a low red blood cell count), which is a common sign of multicentric Castleman disease, and abnormal platelet counts. Having either thrombocytosis (a high platelet count) or thrombocytopenia (a low platelet count) can also indicate multicentric Castleman disease. Your doctor may also test for HIV, which those with HHV-8-associated multicentric Castleman disease often have as well.

Imaging can be helpful to reveal enlarged lymph nodes or an enlarged spleen or liver. Imaging tests a doctor may order include:

Also, doctors usually perform biopsies on lymph nodes that appear to be affected by multicentric Castleman disease.

The only FDA-approved medication to treat multicentric Castleman disease is the anti-IL-6-targeted monoclonal antibody siltuximab (Sylvant). Treatment guidelines suggest this medication as the first-line treatment in people with multicentric Castleman disease who don’t have HIV and HHV-8.

Other treatment options for cases that don’t respond to siltuximab or for people who can’t take it include:

  • rituximab (Rituxan), for people who have HHV-8
  • glucocorticoids
  • cytotoxic chemotherapy

If treatment begins early enough, the outlook for someone with multicentric Castleman disease is generally encouraging.

However, the results of a 2022 study suggest that people with multicentric Castleman disease who are over age 60 years and who have an enlarged spleen have a much poorer outlook than those who don’t have an enlarged spleen.

How long can you live with multicentric Castleman disease?

A 2021 study suggests that with timely treatment, the 5-year survival rate is about 90%. As more doctors become aware of multicentric Castleman disease and can start treatment earlier on in the disease process, life expectancy should continue to improve.

Is multicentric Castleman disease cancer?

Multicentric Castleman disease itself isn’t cancer, but the disease does increase your risk of developing it, especially lymphoma.

Is Castleman disease life threatening?

Castleman disease can be a life threatening disease without prompt and effective treatment. Potential complications of the disease include severe damage to multiple organs and cancer.

Multicentric Castleman disease is a rare but serious disease that raises the risk of severe health complications. Because the cause is usually unknown, there’s little you can do to lower your risk of developing it. However, responding quickly to symptoms can greatly improve your odds of having a positive outcome.

If you notice swollen lymph nodes in your neck, armpit, or groin, or if you have other multicentric Castleman disease symptoms that linger or worsen over several days or a few weeks, seek a medical evaluation as soon as possible. Your symptoms may point to an infection or another condition, but only with an examination and testing can you be sure.