What Is Castleman Disease?
Maybe you've been having an odd sense lately of fullness in your chest or belly. Perhaps you're a little short of breath or not as hungry as usual. Lots of things can cause you to feel this way, and it's easy to shrug off vague symptoms like that. But make sure you check with your doctor, especially if you have HIV. These are also among the signs of Castleman disease.
It's a rare condition that happens when too many cells start to grow in your lymph nodes -- small organs that filter germs. After a while, hard growths start to form there.
Castleman disease isn't cancer. Sometimes, though, it acts a lot like lymphoma, a cancer of the lymph nodes.
There are two types of Castleman disease, and which kind you get makes a big difference in your treatment and the complications you might have.
Unicentric Castleman disease (UCD) only affects a single group of lymph nodes, often in your chest or belly. It's the most common type. You can usually be cured if you have surgery to remove the trouble spots.
The other type is called multicentric Castleman disease (MCD). It affects many lymph nodes in your body. Because it's so widespread, doctors can't remove the problem areas the way they can with UCD. A combination of drugs can keep the disease under control but can't cure it.
If you find out that you have the disease, learn as much as you can about it so you can move forward with confidence when it's time to talk about treatments with your doctor. And don't hesitate to open up to your friends and family about your fears and concerns. They can be a huge source of support while you manage your condition.
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 of 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.
How you feel depends on which type of Castleman disease you have. If you have UCD, you may not have symptoms. If you do notice something, it might be a bulge from the hard growth in the lymph nodes near your neck or under your arms.
When your 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. For instance, a growth in a lymph node in your chest could lead to:
- Trouble breathing
- Wheezing or coughing
- Feeling of fullness in your chest
If the lymph node growth is in your belly, you may have:
- Trouble eating
- Feeling of fullness in your stomach
If you have MCD, you may also have some of the same symptoms as UCD, but on top of that you might notice things like:
- Loss of appetite
- Sweating, especially during the night
- Weak or numb hands or feet
- Weight loss
MCD may also cause swelling or damage to organs like your liver, kidneys, bone marrow, or spleen. You may be more likely to get serious infections because your body can't fight them.
Getting a Diagnosis
If your doctor suspects you have Castleman disease, the first thing they'll likely do is ask you about your medical history. They'll want to know all about your symptoms and if you have any other conditions.
Next, they'll examine you. Since lymph nodes are the prime trouble spots for Castleman disease, they'll check their size and shape.
They'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.
MRI. It uses powerful magnets and radio waves to make pictures of structures, like your lymph nodes.
Ultrasound. 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.
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 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 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 anything while they do 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.
Questions for Your Doctor
If you are diagnosed with Castleman disease, make sure you get all the facts. 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?
You have lots of options. Talk them over carefully with your doctor. Everyone's situation is different, so you'll want to make sure you set up a plan that's right for you. You'll also want to find out about any side effects.
Don't hesitate to get a second opinion from another specialist. Some doctors have more experience than others in treating this rare disease.
If you have the unicentric type of Castleman disease, 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 isn't complicated. You can often go home the same day it's done.
If the lymph node is deep in your belly or chest, your surgeon will need to work harder to get it out. You may need to stay in the hospital for a few days while you recover.
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 for UCD, your doctor may recommend 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's similar to getting an X-ray, and it doesn't hurt. You may need to have these sessions 5 days a week for several weeks.
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 advanced your disease is. It also makes a difference whether you are infected with HIV or HHV-8.
Among the options your doctor may talk about with you are drugs that help stop the inflammation in cells that cause Castleman disease. Some immunotherapy medicines they may suggest are:
- Rituximab (Rituxan)
- Siltuximab (Sylvant)
- Tocilizumab (Actemra)
Some people also take lenalidomide (Revlimid) or thalidomide (Thalomid) to help lower inflammation and ease their symptoms.
Another thing to talk over with your doctor is treatment with corticosteroids. These drugs also reduce inflammation. Prednisone, which is taken as a pill, is often used for Castleman disease.
If your doctor sees signs that you have the HHV8 virus, they may prescribe 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, while others you can take by mouth. Sometimes you'll need to take a combo of different drugs.
A lot of these drugs have side effects, including things like nausea, vomiting, and a higher risk of getting an infection. Always let your doctor know about how you're feeling. They can sometimes make adjustments in doses or prescribe other drugs that can relieve some of problems.
Taking Care of Yourself
Once you've gone through treatment, it's important to have regular checkups with your doctor. They can keep tabs on your health and watch out for signs that the disease is coming back.
If you've been treated for MCD, regular doctor visits can help you stay on the lookout for some complications of the disease. Some people have a greater risk of getting cancers such as Kaposi's sarcoma or lymphoma.
What You Can Expect
If you have the unicentric type of Castleman disease, once your lymph node is removed, you can return to your regular routine. You likely won't get UCD again or feel any symptoms. Your life goes back to normal.
With MCD, the treatment you get may keep it from coming back for a long time. This is called being in remission. But there's always the concern that the disease will come back.
Don't keep your feelings bottled up. Talk to the people who love you about what you're going through. They can help ease your worries and make your life less stressful.
For some people with MCD, the disease may never completely go away, even with treatment. If that happens to you, you may need regular treatment to keep healthy.
Keep in mind that researchers are working all the time to look for new ways to fight Castleman disease. You may want to consider joining a clinical trial, where scientists are trying to find out how well new treatments work. Talk to your doctor if you think you want to take part. You might get medicine or other treatment that can help you. And it can give you the satisfaction of knowing that you're helping to find ways to treat other people in the future.
You don't have to face things alone. Make sure you reach out to family and friends. They can be there when you need some practical care and give you the emotional backing you need.
There may be times that you'll want to talk with someone who's going through the same things you are. That's where a support group can help. Talk with your doctor about how you can connect with others who have Castleman disease.
You can learn more about support groups and get information about the disease by checking the website of Castleman Disease Collaborative Network.