Who discovered Rosai-Dorfman?
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.
Is Rosai-Dorfman disease fatal?
Rosai-Dorfman disease (RDD) is a rare, multisystemic histiocytic disorder, and commonly manifesting as lymphadenopathy in the young male. Abdominal manifestations of RDD are extremely rare.
What is extranodal Rosai-Dorfman disease?
Rosai-Dorfman disease is a rare, idiopathic, benign histiocytic proliferation usually seen in younger patients. Massive lymphadenopathy most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes.
Is Rosai-Dorfman curable?
In many cases, the symptoms of Rosai-Dorfman disease may disappear without treatment (spontaneous remission) within in months or a few years. Clinical observation without treatment is preferred for individuals with Rosai-Dorfman disease whenever possible.
What causes Rosai-Dorfman disease?
The exact cause of Rosai-Dorfman disease is unknown. Possible causes include altered immune responses and infections by agents such as varicella-zoster and other herpetic viruses ; Epstein-Barr; cytomegalovirus; Brucella; and Klebsiella.
Is histiocyte same as macrophage?
DCs, monocytes, and macrophages are members of the mononuclear phagocyte system,2 whereas a histiocyte is a morphological term referring to tissue-resident macrophages. Macrophages are large ovoid cells mainly involved in the clearance of apoptotic cells, debris, and pathogens.
What is the difference between histiocyte and macrophage?
The macrophage is the final stage of development in the monocyte lineage. It is a phagocyte whose roles include the removal of dead and dying tissue and the destruction and ingestion of invading organisms. A histiocyte is a less phagocytic form of a macrophage with fewer lysosomal granules.
Can Rosai-Dorfman disease be cured?
In many cases, the symptoms of Rosai-Dorfman disease may disappear without treatment (spontaneous remission) within in months or a few years. Clinical observation without treatment is preferred for individuals with Rosai-Dorfman disease whenever possible. In many cases, no therapy will be necessary.
What causes Rosai-Dorfman?
Is Rosai-Dorfman autoimmune?
Researchers have suggested that the disorder may be caused by an infectious agent, immunodeficiency, or autoimmunity. Symptoms of Rosai-Dorfman disease develop due to the overproduction and accumulation of histiocytes in the channels (sinuses) that allow for the passage of lymph (sinus histiocytosis).
What is the ICD 10 code for Rosai-Dorfman disease?
External links
| Classification | D ICD-10: D76.3 (ILDS D76.360) MeSH: D015618 DiseasesDB: 31419 |
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| External resources | Orphanet: 158014 |