Castleman Disease

What Is Castleman Disease?

Castleman disease is a rare condition that happens when too many cells grow in your lymph nodes, the small organs that filter out germs. After a while, hard growths form.

Castleman disease isn't cancer. But it can act a lot like lymphoma, a cancer of the lymph nodes.

Doctors use a lot of names to talk about Castleman disease. You might hear it called:

  • Castleman tumor
  • Angiomatous lymphoid hamartoma
  • Angiofollicular ganglionic hyperplasia
  • Angiofollicular lymph hyperplasia
  • Giant lymph node hyperplasia
  • Benign giant lymphoma

 

Types of Castleman Disease

Castleman disease happens in one of two ways.

Unicentric Castleman disease (UCD) affects one group of lymph nodes, often in your chest or belly. It's the most common kind. Surgery to remove those lymph nodes usually cures it.

The other type is called multicentric Castleman disease (MCD). It affects many lymph nodes. Because it's so widespread, doctors can't remove all of the affected nodes the way they can with UCD. Medications can keep it under control.

Microscopic subtypes of Castleman disease

There are also four subtypes, split up by how your lymph tissue looks under a microscope.

  • Hyaline vascular type. This is the most common kind. It’s usually in one place. You probably don’t have many symptoms.
  • Plasma cell type. This is more likely to be multicentric and to cause symptoms.
  • Mixed type. This has areas of both hyaline vascular and plasma cell types.
  • Plasmablastic type. This is usually multicentric and causes symptoms.

 

Castleman Disease Symptoms

How you feel depends on which type of Castleman disease you have. With UCD, you may not have symptoms. Or you might notice a bulge from the hard growth in the lymph nodes near your neck or under your arms.

When UCD causes growths in the lymph nodes in your chest or belly, you may not feel the swelling. But those enlarged areas might bring on other symptoms. A growth in a lymph node in your chest could lead to:

If the lymph node growth is in your belly, you may have:

  • Trouble eating
  • Feeling of fullness in your stomach

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If you have MCD, you may have some of the same symptoms as UCD. You might also notice things like:

MCD can also cause swelling or damage to things like your liver, kidneys, bone marrow, or spleen. You might have low levels of red blood cells (hemolytic anemia) or large amounts of things called immunoglobulins in your blood (your doctor might call this hypergammaglobulinemia). You may be more likely to get serious infections because your body can't fight them.

Castleman Disease Causes

Castleman disease affects the same numbers of males and females. It can happen at any age.

The cause isn’t clear. It might involve problems with your immune system, your body's main defense against germs. It could also be tied to changes in your genes.

If you have HIV, you may have a higher risk of getting multicentric Castleman disease. Because your immune system is weak, you're more likely to have problems from another virus called HHV-8. Scientists think that’s linked to the growth of too many cells in your lymph nodes.

Castleman Disease Diagnosis

Your doctor will ask about your medical history, your symptoms, and whether you have any other conditions.

Because lymph nodes are the main trouble spots for Castleman disease, they'll check their size and shape.

You might have imaging tests like:

  • CT scan. A powerful X-ray makes detailed images of things inside your body.
  • MRI. Strong magnets and radio waves make pictures of things in your body, like your lymph nodes.
  • Ultrasound. Sound waves create an image of your organs.

You'll also have a blood test to look for signs of inflammation.

Your doctor will want to look at a piece of your lymph node under a microscope. You’ll have a procedure called a biopsy to remove a bit of tissue.

If the lymph node is near the surface of your skin, your doctor can sometimes remove all of it easily. If they need to check just a small piece, they'll use a special needle to get it out. Either way, you'll get medicine that numbs the area so you won't feel it.

If the lymph node is in your chest or belly, you'll need medicine that puts you to sleep during the biopsy.

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Questions for Your Doctor

If you’re diagnosed with Castleman disease, you might have lots of questions. Some things you can ask your doctor are:

  • What type of Castleman disease do I have?
  • Are my other health problems or medications causing the condition?
  • Will my symptoms get worse if I don't treat it?
  • Will I need surgery?
  • If I have surgery to remove the swollen lymph node, am I cured?
  • Are there drugs to treat it?
  • Will I get side effects from my treatment?
  • How will my disease affect my body?
  • What can I do to avoid infections?

 

Castleman Disease Treatment

Your treatment will depend on many things, including the type of Castleman disease you have. Talk to your doctor about a plan that's right for you.

You can also get a second opinion from another specialist. Some doctors have more experience than others in treating this rare disease.

Unicentric Castleman disease

If you have the unicentric type, you may need surgery to remove your swollen lymph node. When the node is in an easy-to-reach place, like your armpit, the procedure is simple. You can often go home the same day.

If the lymph node is deep in your belly or chest, it will be harder to get out. You may need to stay in the hospital for a few days after surgery.

Either way, once you take out the lymph node, you're usually cured. Your chances of getting Castleman disease again are very low.

Instead of surgery, you might have radiation therapy to destroy the lymph node. For this treatment, you lie on table while a technician uses a machine that aims high-energy beams at parts of your body. It doesn't hurt. You may need to have these sessions 5 days a week for several weeks.

Multicentric Castleman disease

If you have MCD, you'll need treatment that works throughout your body, because the disease has spread to many lymph nodes.

The kind of therapy you get depends partly on how far along your disease is and whether you have HIV or HHV-8.

Your doctor might give you drugs that help stop the inflammation in cells that cause Castleman disease. They might prescribe immunotherapy medicines like:

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Lenalidomide (Revlimid) or thalidomide (Thalomid) can also help lower inflammation and make you feel better.

Corticosteroids can reduce inflammation. Prednisone, which is taken as a pill, is often used for Castleman disease.

If your doctor sees signs of the HHV-8 virus, they may give you antiviral drugs to help fight it.

Even though Castleman disease isn't cancer, chemotherapy can sometimes help treat MCD. Some chemo drugs are injected into your vein, but others you can take by mouth. Sometimes, you'll need to take a mix of drugs.

The medications can have side effects, including upset stomach, vomiting, and a higher risk of getting an infection. Talk to your doctor if the effects are causing problems. They might be able to change your dose or your medication.

Once you've had treatment, it's important to have regular checkups. Your doctor can keep tabs on your health and watch for signs that the disease might be coming back.

Castleman Disease Complications

Castleman disease can increase your risk of cancers such as Kaposi sarcoma or lymphoma. Because it affects your lymph nodes, which are part of your immune system, MCD might hurt your body’s ability to fight infections. These can turn serious or even deadly.

Castleman Disease Outlook

If you have UCD, you can return to your routine after your lymph node is removed. You probably won't have any more problems.

With MCD, treatment may keep it from coming back for a long time. This is called being in remission.

For some people, MCD never goes away completely. You may need regular treatment.

Talk to your loved ones about what’s happening. They can help ease your worries and make your life less stressful.

You might also want to talk with someone who's going through the same things you are. That's where a support group can help. Ask your doctor for recommendations, or visit the website of the Castleman Disease Collaborative Network.

Researchers are looking for new ways to fight Castleman disease. You may want to join a clinical trial, in which scientists study how well new treatments work. Talk to your doctor if you think you want to take part. You might get medicine or other treatment. And it might ease your mind to know that you're helping find ways to treat other people.

WebMD Medical Reference Reviewed by Sabrina Felson, MD on December 05, 2019

Sources

SOURCES:

Castleman Disease Collaborative Network.

Fajgenbaum, D. Blood, March 12, 2014.

American Cancer Society: "What Is Castleman Disease?" "Treatment of Multicentric Castleman Disease."

National Organization for Rare Disorders: "Castleman's Disease."

Chelsea and Westminster Hospital, National Health Services UK: "Understanding Castleman's Disease."

UpToDate: "Multicentric Castleman's disease."

Radiology: “Angiography of angiomatous lymphoid hamartoma (Castleman tumor) and a suggested pathogenesis.”

National Center for Advancing Translational Sciences Genetic and Rare Diseases Information Center: “Castleman disease.”

Mayo Clinic: “Castleman disease.”

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