Cleveland Clinic doctor feeling lymph nodes in the neck of his patient.

Castleman Disease

You thought you had a lingering flu. But now you’ve learned it could be a rare immune and blood condition you’ve never heard of.

Being diagnosed with Castleman disease can leave you with a lot of questions. What causes it? How’s it treated? What does the future look like? There are also several types and subtypes of this rare condition, and each requires different treatments. So, the first important step is finding an expert healthcare team that can provide the right diagnosis, guidance, support and answers.

While this condition is rare, it’s not uncommon for Cleveland Clinic healthcare providers. We have a team that regularly diagnoses and treats Castleman Disease. We focus on personalizing treatment to your unique diagnosis and needs, helping you move forward through each step.

Why Choose Cleveland Clinic for Castleman Disease Care?

Comprehensive treatment:

Castleman disease is complex, so you’ll have a care team of providers from many specialties. They all work closely together to give you highly personalized care with the best results.

Specialized knowledge:

Castleman disease is rare, but our team is one of the few in the country that treats it regularly. We have years of experience treating this condition, including unicentric Castleman disease (UCD) and all subtypes of multicentric Castleman disease (MCD). Since many conditions — like lymphoma or myeloma, autoimmune conditions or infections — can mimic CD, it’s important to rule out these conditions before making a diagnosis of CD.

Innovation and research:

We have expertise in managing all types of Castleman disease including complications and treatment side effects. We’re also part of the Castleman Disease Collaborative Network (CDCN), a global initiative that provides patients and their families with various kinds of support and opportunities to participate in clinical trials and research.

Patient-centered care:

Everyone with Castleman disease has their own story. We’re here to listen to yours so we can make sure treatment fits your diagnosis, lifestyle and needs. This includes caring for you mentally, emotionally and physically. Meet our team.

Virtual visits:

When you don’t feel well, getting to your provider’s office for an appointment can seem like too much. Don’t worry. Virtual visits are a convenient way to get the same great care from the comfort of home. All you need is an internet connection, and you can connect with your smartphone, computer or tablet.

National recognition:

Cleveland Clinic is a trusted healthcare leader. We're recognized in the U.S. and throughout the world for our expertise and care.

Diagnosing Castleman Disease at Cleveland Clinic

Castleman disease makes your immune system go into hyperdrive, causing abnormal cell growth in your lymph nodes and surrounding tissues. We don’t know what makes this happen, but we think that in some cases, it’s linked to viral infections like HHV-8 or HIV, as part of a syndrome or due to abnormal regulation of your immune system.

There are two types of Castleman disease:

Unicentric Castleman disease (UCD): Also called localized Castleman disease, this type affects one or more lymph nodes in a single area (region) of your body. The cause of UCD is unknown (idiopathic).
Multicentric Castleman disease (MCD): This type causes enlarged lymph nodes in multiple areas of your body. There are three subtypes — idiopathic multicentric CD (iMCD), POEMS-associated MCD and HHV-8-associated MCD. iMCD can be further classified to the following subtypes: iMCD-TAFRO, iMCD-NOS and iMCD-IPL.

In addition to swollen lymph nodes, Castleman disease can cause fatigue, flu-like symptoms and night sweats. In serious cases, it can cause multiple organ failure, which can be fatal. That’s why it’s important to work with healthcare providers who are experienced and skilled in diagnosing and treating it.

What to expect at your first visit

Your first visit is a time for you and your provider to get to know each other. We’re here to help ease your fears and guide you through every step of confirming your diagnosis. We’ll also want to hear what’s been happening in your words. Your provider may ask about:

What kind of symptoms you have.
How long you’ve had these symptoms.
If you’ve noticed any swelling in your neck, groin or underarms.
If any symptoms are getting worse.
How your symptoms have been affecting your life.
If immune conditions or cancer runs in your family.

During this visit, you’ll also have a physical exam so your provider can check for swollen lymph nodes and other signs of Castleman disease, like an enlarged spleen or liver, or swelling in your feet, ankles or belly (abdomen). This exam can tell us a lot about your symptoms and overall health. More testing will help us confirm a diagnosis. These tests may include:

Urinalysis and blood tests

A urinalysis and blood tests help rule out other health conditions that look like Castleman disease. We use these tests to check for abnormalities in blood counts (too little or too much), abnormal kidney or liver function, inflammation, normal proteins (too low), abnormal proteins and identify any infections.

Imaging tests

Your provider may recommend imaging tests to check for an enlarged liver, spleen and lymph nodes, damaged tissue and other signs of Castleman disease inside your body. You may have:

Lymph node biopsy

A lymph node biopsy is required to diagnose Castleman disease. We’ll remove a tissue sample from one of your lymph nodes, or the entire lymph node. Lymph nodes are located throughout your body, including your groin, underarms, neck and abdomen. We use local or general anesthesia to keep you comfortable during the biopsy.

We send the sample to our lab where our pathologists look at it under a microscope. This lets us rule out other conditions. And it helps us start planning your personalized treatment. Each type of Castleman disease responds to different treatment, so we collect as much information as we can at the lab.

Second opinions for Castleman disease

Hearing you have a rare condition like Castleman disease can be a shock. But even so, you want to make sure you have the best team of healthcare providers on your side. At Cleveland Clinic, we encourage second opinions. We want you to feel comfortable with the care you receive and know you’re in capable hands. And we make sure to offer priority scheduling for anyone who requests a second opinion. That way we can dive in, confirm a diagnosis and quickly start treatment.

Meet Our Castleman Disease Team

Typically, you’ll work with providers specializing in hematology and oncology. But you may see other providers, too. It all depends on how Castleman disease affects you. We choose all the members of your care team based on your unique needs and goals, and each one has experience treating Castleman disease. Depending on your diagnosis, your team may include:

Hematologists.
Oncologists.
Rheumatologists.
Surgeons.
Radiologists.
Pathologists.
Nurse practitioners.
Physician assistants.
Care coordinators (providers who keep you in the loop about your treatment plan and advocate on your behalf).
Social workers.

Providers Who Treat Castleman Disease

Locations

Our healthcare providers see patients at convenient locations in Northeast Ohio.

Treating Castleman Disease at Cleveland Clinic

Once your care team confirms your diagnosis, they’ll walk you through each step of your personalized treatment plan, going over what you can expect. Treatment can vary depending on what type of Castleman disease you have.

Treating unicentric Castleman disease (UCD)

If you have UCD, you’ll have surgery to remove any affected lymph nodes. Depending on their size and location, you may have immunotherapy or radiation therapy before surgery. This can shrink the growths and make them easier to remove.

If your provider can’t remove the tumor and you don’t have symptoms, we may keep an eye on the tumor instead of treating it immediately. If you do have symptoms but surgery isn’t an option, we may recommend radiation therapy, immunotherapy or other treatments that are typically only used for multicentric Castleman disease.

Treating multicentric Castleman disease (MCD)

MCD is harder to treat because it’s widespread throughout your body. We don’t usually recommend radiation or surgery. We plan treatment based on how severe your MCD is and if it’s connected to HHV-8. We may recommend:

Corticosteroids to relieve symptoms and reduce inflammation.
Chemotherapy to slow cell overgrowth in your lymph nodes.
Immunotherapy: Siltuximab (Sylvant^®) to block proteins that fuel abnormal cell growth.
Antiviral drugs to treat HIV or HHV-8 infections.

Taking the Next Step

Having a rare condition like Castleman disease might be challenging, but that doesn’t mean it’s not treatable. Especially when you have a healthcare team that’s highly skilled and experienced in caring for people with this uncommon condition. We offer personalized, compassionate care that keeps your unique needs and goals in mind. Using the latest tests and treatments, we’ll work to make sure you feel good physically and emotionally as you navigate this journey.