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Rosai-Dorfman Disease

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You might brush off your constant tiredness or swollen glands as a passing virus. But what does it mean when they won’t go away?

Sometimes, symptoms for rare blood conditions like Rosai-Dorfman disease (RDD) often look and feel like an everyday illness. So, it can be a real surprise to get this diagnosis. While it might explain why you haven’t felt great, you’re probably wondering what this all means — and what your future might look like.

Cleveland Clinic’s expert healthcare providers have answers and the compassionate care you need. As you begin to wrap your head around this new diagnosis, we’re here to listen to you and answer your questions. We want to understand your story and concerns so we can find the best treatment for your needs.

Why Choose Cleveland Clinic for Rosai-Dorfman Disease Care?

Comprehensive treatment:

We offer every available treatment for RDD — surgery, targeted therapy, immunosuppressive medications, chemotherapy and radiation. And we’re quick — our providers do their best to see anyone with confirmed or suspected RDD from anywhere in the country within five business days.

Personalized care:

RDD affects each person differently. Cleveland Clinic’s experts recommend treatments based on your individual symptoms and personal needs. We may take a “wait-and-watch” approach. Or use minimally invasive therapies to help you manage symptoms. We recommend more intense treatments and surgical approaches only when you need them.

Skilled, collaborative providers:

Rosai-Dorfman disease often requires care from providers across different specialties. Your care team of experts works together to create a care plan based on how RDD affects you. We’re here to help every step of the way, from diagnosis to regular follow-up care. Meet our team.

Innovation and research:

Cleveland Clinic’s active research program helps us improve diagnosis, treatment and your quality of life if you have a blood condition. Clinical trials may give you access to the latest treatment not widely available.

Virtual visits:

Not feeling great? Rather stay curled up on the couch than drive to an appointment? Virtual visits are a convenient alternative to seeing your provider in person. You’ll get the same expert care but from the comfort of your home.

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. Our blood diseases program is listed as a center of excellence by the National Organization of Rare Diseases (NORD).

Diagnosing Rosai-Dorfman Disease at Cleveland Clinic

Rosai-Dorfman disease is a benign (not cancerous) blood condition. It starts when histiocytes, a type of white blood cells, start growing out of control. There are two types:

Classic (nodal) Rosai-Dorfman disease: Causes massive enlargement (swelling) of lymph nodes in one or more areas of your body, particularly in your neck.
Extranodal Rosai-Dorfman disease: Can affect tissue other than lymph nodes, such as your brain, eyes, kidneys, bones, skin and others.

Diagnosing Rosai-Dorfman disease isn’t always easy. Symptoms can vary from person to person. You may have none and learn you may have RDD through routine bloodwork or imaging done for some other reason (such as mammogram or CT scan) or after a colonoscopy. And common symptoms — swollen lymph nodes, fever, fatigue and night sweats — are often like those from other conditions. RDD can also happen with certain cancers like lymphomas or rheumatologic conditions such as lupus (systemic lupus erythematosus). That’s why it’s important to work with providers experienced in diagnosing and treating RDD.

What to expect at your first visit

When you come to your first appointment with us, you may feel a little stressed — and have many questions. That’s why your provider will start off by getting to know you. They’ll take time to listen to your concerns, answer your questions and even ask some of their own, like:

What symptoms do you have?
How long have you had these symptoms?
How are your symptoms affecting your life?
Have you had testing before this appointment?
What did you learn from your test results?
Have you been diagnosed with other health conditions?
Does anyone in your family have a blood condition?

You’ll also have a thorough physical exam. Your provider will look for signs of RDD, like swollen lymph nodes or skin growths.

Testing for Rosai-Dorfman disease

Your provider will also order a series of tests to help them rule out other conditions and to confirm a Rosai-Dorman disease diagnosis. You may have:

Complete blood count.
Comprehensive metabolic panel.
X-ray.
Ultrasound.
CT scan.
Bone scan.
MRI.
Whole body FDG-PET scan.
Biopsy.
Genomic analysis.

Meet Our Rosai-Dorfman Disease Team

Cleveland Clinic has all the providers you need to manage your RDD. A hematologist will lead your personalized care team. You may also see:

Your care team works together to confirm a diagnosis and help you move forward with your personalized RDD treatment.

Provider Who Treats Rosai-Dorfman Disease

Locations

Our healthcare providers see patients at Cleveland Clinic Main Campus.

Treating Rosai-Dorfman Disease at Cleveland Clinic

We personalize your Rosai-Dorfman disease treatment around how and where it affects you — and how it’s progressed. We’ll use your test results as a guide when crafting your care plan. We’ll also consider your age, overall health and lifestyle.

Rarely, RDD goes away on its own. You may only need “watchful waiting.” This means we won’t do treatment. Instead, we’ll keep an eye on your symptoms. And if they get worse, or new ones arise, we may recommend one or more treatments, including:

Medications: Corticosteroids like prednisone (Deltasone^®, Predone^®, Sterapred^®) can reduce swelling and inflammation.
Surgery: We may remove RDD-related growths that cause pressure, breathing or nerve problems.
Radiation therapy: Radiation therapy can help shrink enlarged lymph nodes if surgery isn’t possible. Or if surgery didn’t work.

Systemic therapy

Your care team may recommend treatments like these that can attack histiocytes throughout your body:

Immunotherapy: Helps your immune system destroy histiocytes.
Targeted therapy: Depending on the particular gene defect found during genomic analysis, several targeted therapy options are available, including an oral drug cobimetinib (Cotellic^®) which can be effective.
Chemotherapy: We may use this to treat severe RDD or if other treatments don’t improve symptoms.

Living with Rosai-Dorfman Disease

While RDD may respond to treatment, it sometimes can come back (recur) — so RDD has to be monitored the rest of your life. But we’ll track your health with regular follow-up visits and testing either at Cleveland Clinic or in coordination with your local providers. And we’ll ask you to talk openly and honestly with your provider about any new symptoms. This helps us start treatment again as quickly as possible.

Taking the Next Step

When those swollen lymph glands are more than a passing infection, you can count on Cleveland Clinic to help you take the next step. Our compassionate team answers your questions about having (and treating) Rosai-Dorfman disease. We guide you through personalized treatment and follow-up care so you can feel like yourself again.