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Hello I was wondering if anybody here has ever heard of this autoimmune disease. If so, what treatment worked for you. Thank you
Hello, I just had an inguinal lymph node biopsy and the results came out as Sinus Histiocytosis/Rossi Dorfman Disease. I was wondering if anybody here has ever had this diagnosis. What treatment plan worked for you.. Please help
Hi @roger191, you'll notice that I merged the 2 discussions that you started into one discussion that appears in both the Autoimmune Diseases group and the Blood Cancers & Disorders group simultaneously. Given that Rosai-Dorfman Disease is rare, it will be helpful to have all the messages in one central place.
Personally, I am unfamiliar with Rosai-Dorfman Disease (sinus histiocytosis with massive lymphadenopathy – SHML) and found more information about the condition on the National Organization for Rare Disease (NORD) website here:
While it is difficult to hear that you have a rare condition, I wonder if there is a bit of relief that you got a diagnosis and can move forward with treatment. Did it take a long time to get a diagnosis? What treatment plan is being suggested for you?
@colleenyoung thank you. It has taken over six months for me to get that diagnosis. I'm yet to see any Dr, my appointment was set for next month. As much as it's been a relief, the pain has been somewhat unbearable because i have also been having chest pain. I was just curious to know if its related. Thanks for the link, let me read more about it
Roger, I hope the article will help you prepare for your appointment next month. Are you seeing a hematologist?
You may be interested in reading this discussion where members offer tips about starting with a new specialist.
> Your Tips on How to Get Off to the Best Start with a New Specialist https://connect.mayoclinic.org/discussion/your-tips-on-how-to-get-off-to-the-best-start-with-a-new-specialist/
i'd also like to bring @dazlin @peabody88 and @ecdhope who have also been investigating causes of histiocyte level changes and Rosai-Dorfman disease. They may also be able to help create a list of questions to ask.
Please keep us posted.
Thank you so much for your help and for the articles. Yes, I will be seeing a hematologist. I will keep you posted
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Roger191, a friends father was diagnosed with Rosai Dorfmans disease. He is literally on deaths door without appropriate care in his hometown. Did you get help from a good doctor that you could recommend?
Hello. I was diagnosed with Rosia-Dorfman disease in August and have found no one to help me. I was placed on blood thinners and the hematologists who was supposed to help with the disease canceled our first appointment the night before and said she couldn’t not help me. Now I am trying to find a doctor who can help me figure out how to come off the blood thinners and what I wed to do about the blood clots then Rosia-Dorfman is causing. And help or recommendations are appreciated. Thank you!
@lollya74 @eal, Welcome to Mayo Clinic Connect. I ran across an article that may be helpful by searching on Google Scholar (https://scholar.google.com/).
The article is a special report done on June 28th, 2018 – Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease: https://ashpublications.org/blood/article/131/26/2877/39185/Consensus-recommendations-for-the-diagnosis-and
The report includes information gathered by doing an English-language search of PubMed for RDD-related literature from 1965 until 2018. The treatment section of the report includes treatment information that may be helpful when you talk with your hematologist. Here is the Introduction taken from the report:
"Rosai-Dorfman-Destombes disease (RDD) is a rare non–Langerhans cell histiocytosis (LCH) first described in 1965 by a French pathologist, Pierre Paul Louis Lucien Destombes, who reported 4 children and young adults with lymphadenopathy and sinus histiocytosis upon histologic analysis.1 Four years later, Juan Rosai and Ronald Dorfman analyzed 34 cases of the same entity under the name sinus histiocytosis with massive lymphadenopathy.2 Characteristic lesional histiocytes are S100+, CD68+, and CD1a− and demonstrate variable frequency of emperipolesis. Historically, RDD has been considered a self-limited disorder of unknown etiology, although few patients have poor outcomes.3 Patients with classical RDD present with bilateral cervical lymphadenopathy, but 43% of patients with RDD present with extranodal disease.3 RDD is a heterogeneous entity that can occur as an isolated disorder or in association with autoimmune, hereditary, and malignant diseases. Because of the wide clinical spectrum of RDD and the consequent variety of specialists evaluating and treating such patients, there is a need for an evidence-based approach to the evaluation and treatment of this protean condition."
There is a PDF button on the side of the page that allows you to print or download the 14 page report that might be helpful for your doctor.
Have you considered seeking help at a major teaching hospital or the Mayo Clinic?
Thank you so much. Yes I have considered seeking help at a major teaching hospital or Mayo but I have no idea where to start. My doctors here have basically abandoned me so I am trying to understand the disease, try to figure out how to fix this, and work full time. I have wonderful health insurance, and even they are having trouble finding someone to help me.
Hi @eal, I can't imagine how difficult it is to deal with such a rare disease and still be working full time. If you would like to seek help from Mayo Clinic, you can contact one of the appointment offices. The contact information for Minnesota, Arizona and Florida can be found here http://mayocl.in/1mtmR63. There is a discussion that you might find helpful on getting your first appointment here: https://connect.mayoclinic.org/discussion/getting-your-first-appointment/
I just found this article from Feb 2020 that may help you learn a little more about the disease.
– Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease: https://haematologica.org/article/view/9519
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!
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