Rosai-Dorfman disease

Common Name(s)

Rosai-Dorfman disease

Rosai-Dorfman disease was first described by Rosai and Dorfman in 1969.  It is a benign disease which is characterized by over-production and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body, most often those of the neck (cervical).  Other lymph node groups may also be involved and, in some cases, abnormal accumulation of histiocytes may occur in other areas of the body (extranodal). The cause of this condition remains unknown, although altered immune responses and infectious agents may play a role. SHML is a self-limited and seldom life-threatening disease which commonly does not require therapy.
 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Rosai-Dorfman disease" for support, advocacy or research.

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Histiocytosis Association

The Histiocytosis Association is dedicated to raising awareness about histiocytic disorders, providing educational and emotional support, and funding research leading to better treatments and a cure.

Last Updated: 30 Oct 2012

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Rosai-Dorfman disease" for support, advocacy or research.

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Histiocytosis Association

The Histiocytosis Association is dedicated to raising awareness about histiocytic disorders, providing educational and emotional support, and funding research leading to better treatments and a cure.

http://www.histio.org

Last Updated: 30 Oct 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Rosai-Dorfman disease" returned 93 free, full-text research articles on human participants. First 3 results:

Extra Nodal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy : An Atypical Ocular Presentation.
 

Author(s): Eesha Shukla, Anjali Nicholson, Anamika Agrawal, Darshana Rathod

Journal: Head Neck Pathol. 2016 Sep;10(3):414-7.

 

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder, presenting as painless bilateral cervical lymphadenopathy, with systemic symptoms. Extra nodal manifestations have been reported in ...

Last Updated: 4 Aug 2016

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Rosai-Dorfman Disease Harboring an Activating KRAS K117N Missense Mutation.
 

Author(s): Vignesh Shanmugam, Elizabeth Margolskee, Michael Kluk, Tamara Giorgadze, Attilio Orazi

Journal: Head Neck Pathol. 2016 Sep;10(3):394-9.

 

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare histiocytic proliferation that is generally considered to be reactive with a benign clinical course. The etiology of RDD is very poorly understood. Recent studies have shown frequent BRAF, NRAS, ...

Last Updated: 4 Aug 2016

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MRI and CT findings of isolated intracranial Rosai-Dorfman disease in a child.
 

Author(s): Ayse G Alimli, Cigdem Oztunali, Oznur L Boyunaga, Selma Pamukcuoglu, Arzu Okur, Alp O Borcek

Journal: Neuroradiol J. 2016 Apr;29(2):146-9.

 

Isolated intracranial Rosai-Dorfman disease (RDD) is extremely rare in pediatric patients. We present the case of a 22-month-old boy whom had isolated intracranial RDD involvement. To our knowledge, a parieto-occipital regional involvement without a dural tail sign has not been previously ...

Last Updated: 15 Mar 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Rosai-Dorfman disease" returned 18 free, full-text review articles on human participants. First 3 results:

Isolated localization of Rosai Dorfman disease as renal mass: a case report and review of literature.
 

Author(s): Aziz El Majdoub, Aziza El Houari, Laila Chbani, Hinde El Fatemi, Abdelhak Khallouk, Moulay Hassan Farih

Journal:

 

We report a rare case of an elderly woman presented with right renal mass with invasion of renal vein and several small lymphadenopathy in the hilar area. The diagnosis of kidney cancer is suspected and the patient underwent open radical nephrectomy, surrenalectomy and lymphadenectomy ...

Last Updated: 19 Sep 2016

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Soft tissue Rosai-Dorfman disease in child: A case report and literature review.
 

Author(s): Yunlan Xu, Bingqiang Han, Jie Yang, Jing Ma, Ji Chen, Zhigang Wang

Journal: Medicine (Baltimore). 2016 Jul;95(29):e4021.

 

Rosai-Dorfman disease was commonly characterized as massive, painless, bilateral, symmetric cervical lymphadenopathy, with fever, leukocytosis, and elevated sedimentation rate. However, soft tissue Rosai-Dorfman disease (STRDD) is a rare benign tumor.

Last Updated: 22 Jul 2016

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Rosai-Dorfman Disease Isolated to the Thoracic Epidural Spine.
 

Author(s): Benjamin Kozak, Jason Talbott, Alina Uzelac, Bhavya Rehani

Journal:

 

Rosai-Dorfman disease is a rare benign histiocytic disease that infrequently presents in the spine. We report a case of Rosai-Dorfman disease isolated to the epidural thoracic spine in a 26-year-old male. To our knowledge, this is the 15th reported case of isolated spinal disease ...

Last Updated: 2 Jun 2016

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

A Study of Memory, Thinking, and Brain Imaging in Adults With Histiocytosis
 

Status: Recruiting

Condition Summary: Histiocytosis

 

Last Updated: 20 Apr 2017

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International Rare Histiocytic Disorders Registry (IRHDR)
 

Status: Recruiting

Condition Summary: Rare Histiocytic Disorders (RHDs); Juvenile Xanthogranuloma (JXG); Reticulohistiocytoma (Epithelioid Histiocytoma); Xanthoma Disseminatum (XD); Multicentric Reticulohistiocytosis (MRH); Systemic Juvenile Xanthogranuloma; Erdheim-Chester Disease (ECD); Multi-system Rosai-Dorfman Disease (RDD)

 

Last Updated: 25 Apr 2016

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