Castleman disease

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Kemas kini Tarikh 12/05/2020 . 5 minit bacaan
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What is Castleman disease?

Castleman disease involves an overgrowth (proliferation) of cells in your body’s disease-fighting network (lymphatic system). Also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, Castleman disease can occur in a localized (unicentric) or widespread (multicentric) form.

Treatment and outlook vary, depending on the type of Castleman disease you have. The localized type can usually be successfully treated with surgery.

Sometimes associated with HIV infection, multicentric Castleman disease can be life-threatening. Multicentric Castleman disease is also associated with other cell-proliferation disorders, including cancer of the lymphatic system (lymphoma), Kaposi’s sarcoma and POEMS syndrome.

How common is Castleman disease?

Castleman disease is a rare disorder. Please discuss with your doctor for further information.


What are the symptoms of Castleman disease?

There are two basic types of Castleman disease:

  • Unicentric Castleman disease. This localized form of the disease affects only a single gland (lymph node) in your lymphatic system.
  • Multicentric Castleman disease. This type affects multiple lymph nodes and lymphatic tissues, and can severely weaken your immune system.

Multicentric Castleman disease can be further classified as:

  • Multicentric Castleman disease without POEMS syndrome
  • Multicentric Castleman disease with POEMS syndrome that involves areas of abnormal bone (osteosclerotic lesions)
  • Multicentric Castleman disease with POEMS syndrome without osteosclerotic lesions

Unicentric Castleman disease

Many people with unicentric Castleman disease don’t notice any signs or symptoms. The diseased lymph node is usually located in the chest, neck or abdomen. When signs and symptoms are present, they may include:

  • A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
  • An enlarged lump under the skin in the neck, groin or armpit
  • Unintended weight loss
  • Less commonly, fever, night sweats and weakness

Multicentric Castleman disease

Most people with multicentric Castleman disease experience:

  • Fever
  • Night sweats
  • Fatigue and weakness
  • Loss of appetite
  • Unintended weight loss
  • Enlarged lymph nodes, usually around the neck, collarbone, underarm and groin areas
  • Enlarged liver or spleen

Other, less common symptoms include:

  • Nerve damage in the hands and feet that leads to numbness (peripheral neuropathy)
  • Skin rash

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.


What causes Castleman disease?

It’s not clear why you get Castleman disease. Part of it seems to be connected to problems with the immune system — your body’s main defense against germs.

If you have HIV, the virus that causes AIDS, you may have a higher risk for getting the multicentric form of Castleman disease. Your immune system is weak, and you’re more likely to get infected with another virus called HHV8. Scientists aren’t sure why or how, but this virus seems to be linked in some way to the growth of too many cells in the lymph nodes.

Risk factors

What increases my risk for Castleman disease?

Castleman disease can affect anyone. 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 only known risk factor for Castleman disease appears to be having HIV/AIDS.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Castleman disease diagnosed?

If your doctor suspects you have Castleman disease, the first thing he’ll likely do is ask you about your medical history. He’ll want to know all about your symptoms and if you have any other conditions.

Next, he’ll examine you. Since lymph nodes are the prime trouble spots for Castleman disease, he’ll check their size and shape.

He’ll also do some scans of your body. You might need to get one of these:

  • CT scan. It’s a powerful X-ray that makes detailed images of things inside your body.
  • It uses powerful magnets and radio waves to make pictures of structures, like your lymph nodes.
  • It uses sound waves to create an image of your organs.

You’ll also take a blood test to see if you have signs of inflammation in your body that can point to Castleman disease.

Your doctor will want to take a look at a piece of your lymph node under a microscope. You’ll get what’s called a “biopsy” — a procedure to remove a tiny piece of your tissue.

There are several different ways to do this. If the lymph node is near the surface of your skin, your doctor can sometimes remove all of it easily. If he needs to check just a small piece, he’ll use a special needle to get it out. Either way, you’ll get medicine that numbs the area so you won’t feel anything while he does it.

If the lymph node is in your chest or belly, your doctor can also do a biopsy, but you’ll need medicine that puts you under while it’s happening.

How is Castleman disease treated?

Treatment depends on the type of Castleman disease you have.

Unicentric Castleman disease

Unicentric Castleman disease can be cured by surgically removing the diseased lymph node. If the lymph node is in your chest or abdomen — which is often the case — major surgery may be required.

If surgical removal isn’t possible, medication may be used to shrink the lymph node. Radiation therapy also may be an effective way to destroy the affected tissue.

You’ll need follow-up exams, including imaging, to check for relapse.

Multicentric Castleman disease

Surgery usually isn’t an option for multicentric Castleman disease because of the number of lymph nodes involved. However, surgery to remove an enlarged spleen may be an option to help ease symptoms.

Treatment generally involves medications and other therapies to control cell overgrowth. Specific treatment depends on the extent of your disease and on whether you have HIV or HHV-8 infection or both.

The options include:

  • Monoclonoal antibodies, to block the action of the IL-6 protein that contributes to cell overgrowth. Your doctor may recommend initial treatment with a monoclonal antibody, such as siltuximab (Sylvant), if you don’t have organ damage or HIV or HHV-8 infection.
  • Chemotherapy, to slow overgrowth of lymphatic cells. Your doctor may recommend adding chemotherapy if the disease doesn’t respond to monoclonal antibodies or if you have organ failure.
  • Corticosteroids, to control inflammation.
  • Antiviral drugs, to block the activity of HHV-8 or HIV if you have one or both of those viruses.
  • Thalidomide (Thalomid), to block the action of the IL-6 protein. Thalidomide is an immune-system modulator that has been shown to be effective at inducing remission in Castleman disease.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Castleman disease?

The following lifestyles and home remedies might help you cope with Castleman disease:

  • Find someone to talk with. You may feel comfortable discussing your feelings with a friend or family member, or you might prefer meeting with a formal support group.
  • Set reasonable goals. Having goals helps you feel in control and can give you a sense of purpose. But choose goals that you can reach.

If you have any questions, please consult with your doctor to better understand the best solution for you.

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