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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How do you compare to others with this condition?

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

Hypercalcemia induced by Rosai-Dorfman disease in a hemodialysis patient: histological evidence of extrarenal calcitriol overproduction.
 

Author(s): Shunsuke Yamada, Mai Uemura, Masanori Tokumoto, Tadashi Nagara, Hideko Noguchi, Minako Hirahashi, Toshiaki Nakano, Kazuhiko Tsuruya, Takanari Kitazono

Journal: Intern. Med.. 2014 ;53(24):2783-7.

 

Rosai-Dorfman disease (RDD) is a rare disorder characterized by the proliferation of histiocytes in the sinus of the affected lymph nodes, which leads to massive lymphadenopathy. RDD usually presents as an increased inflammatory response and lymph node swelling. We herein report the ...

Last Updated: 16 Dec 2014

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No increase in IgG4-positive plasma cells in limbal Rosai-Dorfman disease.
 

Author(s): Saeed AlWadani, Shaun Robinson, Rory Myers, Esen K Akpek, Charles G Eberhart

Journal: Cornea. 2014 Aug;33(8):844-7.

 

Extranodal Rosai-Dorfman disease is a rare benign condition recently reported to sometimes show features of IgG4-related disease. The purpose of this study was to describe the corneal-limbal manifestation of the entity and to investigate whether numerous IgG4-positive plasma cells ...

Last Updated: 8 Jul 2014

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Case for diagnosis. Cutaneous Rosai-Dorfman disease.
 

Author(s): Han Ma, Huaiqiu Huang, Meirong Li, Wei Lai, Chun Lu

Journal: An Bras Dermatol. ;89(1):171-2.

 

Cutaneous Rosai-Dorfman disease is a rare, lymphoproliferative disease. It is benign and self-limited, only involves skin and subcutaneous tissue and typically occurs as histiocyte-rich inflammatory infiltrates, manifesting as erythematous to brown papules, plaques, or nodules, without ...

Last Updated: 14 Mar 2014

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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 12 free, full-text review articles on human participants. First 3 results:

Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment.
 

Author(s): Samir Dalia, Elizabeth Sagatys, Lubomir Sokol, Timothy Kubal

Journal: Cancer Control. 2014 Oct;21(4):322-7.

 

Rosai-Dorfman disease (RDD) is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features.

Last Updated: 14 Oct 2014

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Rosai-Dorfman disease of the pericardium: a case report and review of literature.
 

Author(s): I-Weng Lao, Yun Dong, Jian Wang

Journal:

 

Rosai-Dorfman disease (RDD) is an uncommon histiocytic disease of unknown etiology. It typically presents as massive lymphadenopathy with a predilection for the cervical lymph nodes of children and young adults. However, extranodal involvement is not uncommon and may cause confusion ...

Last Updated: 17 Jul 2014

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Extranodal Rosai-Dorfman disease involving the right atrium in a 60-year-old male.
 

Author(s): Yalan Bi, Zhen Huo, Yunxiao Meng, Huanwen Wu, Jingbo Yan, Yuan Zhou, Xingrong Liu, Lan Song, Zhaohui Lu

Journal:

 

Rosai-Dorfman disease (RDD) involving the cardiovascular system is extremely rare; to our knowledge, there are only 9 cases in the literature. Here, a case of a 60-year-old male with RDD involving the right atrium is presented. A comprehensive literature review was undertaken to summarize ...

Last Updated: 24 Jun 2014

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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: 26 May 2015

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