Rosai-Dorfman Disease (Sinus histiocytosis)

The authors of the February 2020 article that you cite are all members of a Mayo Rochester working group for histiocyte disorders led by Dr. Ronald Go. I have Langerhans cell Histiocytosis which is diagnosed in 1-2 persons per million population. I think that Rosai-Dorfman disease is even more rare. I recommend going to Mayo or to another large center such as Dana Farber, Boston that has doctors with interest in these rare diseases and can help you with a treatment plan.
For an recent video overview of these diseases, go to Texaschildrens.org/departments/Histiocytosis-clinic. Many of these diseases are found primarily in children, but can be diagnosed at any age.

Thank you! I have filled out an appointment request and will wait and see what happens. You have no idea how much I appreciate your help. You have made my day and I greatly appreciate you!

I was hospitalized back in May of this year for a few weeks. After a neck lymphnoid biopsy I was diagnosed with sinus histiocytosis. While in the hospital my body started shutting down I ended up in intubation on life support for a week. I see a oncologist hematologist a primary care doct and a gasternologist. Right now they have me on prednisone and a up keep antibiotic to take every other day. My oncologist Is probably going to be switching me he said to a auto immune drug but he didn't say what kind. I still get swollen lymphnoids I have muscle pain everyday. I'm even loosing my hair sadly. I also work but only part time since I got sick I also have 4 young children so I know how hard it is living with this rare condition. Especially when everyone tells you they don't know enough about it or they can't help. It's hard and stressful.

Welcome @lilrush. I'm tagging @eal to make sure your message to them is seen. Your situation is remarkably similar.

I can't imagine working part time with 4 children and managing sinus histiocytosis. This is all relatively new for you. Were you experiencing symptoms long before you were finally diagnosed? What accommodations have you made in your daily routine that help?

I have no idea how much it feels to find someone who has a similar diagnosis. I was diagnosed in Feb of this year 2025. How much has your disease progressed. Mine is in the sinus and Left eye area.

Welcome, @jackieokoth, I'm tagging @eal @cblowers1 and @lilrush to bring them back to this discussion about Rosai-Dorfman disease (sinus histiocytosis).

Jackie, this is all so recent for you. I can imagine it feels to good to not feel alone and to know there are others out there with a similar diagnosis.

Did you know Mayo Clinic has a team at their Histiocytosis Clinic dedicated to helping adults with histiocytosis disorders, such as Erdheim-Chester disease, Langerhans cell histiocytosis and Rosai-Dorfman disease?
- Histiocytosis Clinic in Minnesota https://www.mayoclinic.org/departments-centers/histiocytosis-clinic-minnesota/overview/ovc-20566040

Jackie, what treatment are you on? How are you doing?

I have Rosai Dorfman and have been taking Cotellic for about a year and a half. My lymph nodes have shrunk in size.

Hello @roger191. This noncancerous disorder is rare but there is a lot more information about it in the past 20 years so don't lose hope. I was diagnosed with Rosai Dorfman in 2004. I had three enlarged lymph nodes removed above my left breast during that time. I also had a large mass on my spine (L3 and L4) that was pushing up against spinal cord. I was given prednisone 40mg for almost 6 months to shrink the mass. The prednisone shrank the mass to the point where I could walk again without pain but didnt totally resolve it. It took years for the mass to resolve on its own (no meds just monitoring it). I was in "remission" for more than 20 years. Fast forward to 2026. My Rosai Dorfman flared up again. This time I developed a mass in my heart that was the size of a small orange between my left and right ventricle. It was discovered because my doctor did a chest x-ray because I had a pneumonia at the time and had difficulty breathing. I was admitted to the hospital for 2 weeks and the doctors ran test after test to figure out what caused this mass. I had tell them that I had Rosai Dorfman 20 years ago and it may have flared up again. I was prescribed prednisone again (60 mg this time) and have been taking it for 6 months. The prednisone shrank the mass by 50% (and all my lymph nodes) but it did not shrink it anymore than that. Now I am starting Cotellic 20mg this week and will be gradually increasing my dosage to 60mg this month. I am praying Cotellic will work. I have read studies that Cotellic has worked for Rosai Dorfman symptoms (enlarged lymph nodes) but I havent seen any studies about its effects on tumors or masses caused by Rosai Dorfman. I have been living with RD for years. I've learned it flares up during very stressful times in my life. Im debating if I need to retire or change jobs now because my workplace is stressful. I have lived through my first RD diagnosis and I will live through this one again. If you have been diagnosed with it dont lose hope. There are more treatments for it and more studies about it. I'm living testament that you can overcome the symptoms and manage it for 20 years.

@michelliot I took Prednisone when I was first diagnosed . I had terrible side effects from it but it significantly shrunk all my lymph nodes but as I was weaning off of it they all became enlarged again. Cotellic has worked well for me. Could only tolerate 20 mg per day Good luck with it.

Hello @robins12. Thank you for the reassurance about taking Cotellic. Im worried about the side effects because of the increased risks of rash and severe sunburn. Did you also have bad GI sides effects like nausea, vomiting, diarrhea, etc?