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

Claudio Doglioni

2021Pathologica20 citationsDOIOpen Access PDF

Abstract

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.

Topics & Concepts

EmperipolesisRosai–Dorfman diseaseHistiocyteHistiocytosisPathologyDiseaseMedicineSinus Histiocytosis with Massive LymphadenopathyErdheim–Chester diseaseHistiocytic Disorders and TreatmentsParvovirus B19 Infection StudiesTumors and Oncological Cases