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 92 free, full-text research articles on human participants. First 3 results:

Rosai-Dorfman disease (RDD) in the paraglottic space: report of a case and review of literature.
 

Author(s): Yu-Ling Ma, Zhuo-Ping Liang, Sheng-En Xu, Zhi-Hui Yang, Yi Peng, Xiao-Qiang Sun, Gang Qin

Journal:

 

Sinus histiocytosis with massive lymphadenopathy is also known as Rosai-Dorfman disease (RDD) and is characterized by painless bilateral cervical lymphadenopathy. In the present case report, a 67-year-old Chinese woman presented with a 3-month history of progressive voice hoarseness, ...

Last Updated: 1 Jan 2016

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Extranodal Rosai-Dorfman Disease as Isolated Lesion of the Tibia Diagnosed by Fine-Needle Aspiration Cytology: A Case Report.
 

Author(s): Jie Xu, Chun-Hua Liu, Yan-Si Wang, Chang-Xian Chen

Journal: Medicine (Baltimore). 2015 Nov;94(47):e2038.

 

Few studies have used fine-needle aspiration cytology for the purpose of isolated skeletal Rosai-Dorfman diseases (RDDs) diagnosis.Herein, we described an extremely rare case of a 56-year-old woman who presented to our hospital with an insidious onset of pain in the right proximal ...

Last Updated: 4 Dec 2015

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Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule.
 

Author(s): Brandon R Litzner, Antonio Subtil, Claudia I Vidal

Journal: Am J Dermatopathol. 2015 Dec;37(12):936-9.

 

Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. ...

Last Updated: 21 Nov 2015

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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 17 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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Tracheobronchial Involvement of Rosai-Dorfman Disease: Case Report and Review of the Literature.
 

Author(s): Louis Boissière, Martine Patey, Olivier Toubas, Juliette Vella-Boucaud, Jeanne-Marie Perotin-Collard, Gaëtan Deslée, Francois Lebargy, Sandra Dury

Journal: Medicine (Baltimore). 2016 Feb;95(7):e2821.

 

Rosai-Dorfman Disease (RDD) is a rare non-neoplastic entity, also known as sinus histiocytosis with massive lymphadenopathy (SHML), characterized by a benign proliferation of histiocytes in lymph nodes. Localized forms of RDD involving the tracheobronchial tree are very rare. There ...

Last Updated: 18 Feb 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

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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