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Rosai-Dorfman disease. A legacy of Professor Rosai that is still not exploited completely

Rosai-Dorfman disease (RDD) is a rare form of non-Langerhans cell histiocytosis described by Rosai and Dorfman in 1969. It is a fascinating disease characterized by accumulation of large, pale histiocytes, frequently showing the emperipolesis phenomenon. The variety of pathological aspects and the s...

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Detalles Bibliográficos
Autor principal: Doglioni, Claudio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Pacini Editore srl 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8720407/
https://www.ncbi.nlm.nih.gov/pubmed/34837097
http://dx.doi.org/10.32074/1591-951X-548
Descripción
Sumario:Rosai-Dorfman disease (RDD) is a rare form of non-Langerhans cell histiocytosis described by Rosai and Dorfman in 1969. It is a fascinating disease characterized by accumulation of large, pale histiocytes, frequently showing the emperipolesis phenomenon. The variety of pathological aspects and the spectrum of different clinical forms were deeply investigated by Prof. Rosai. Despite recent advancements in the dissection of pathogenetic mechanisms of RDD, with the identification of gene mutations in the MAP kinase pathway, several biological and clinical aspects of this disease remains to be elucidated: this is one of the Prof. Rosai’s legacies.