Doglioni Claudio
University Vita-Salute San Raffaele, Milano, Italy.
Pathologica. 2021 Oct;113(5):388-395. doi: 10.32074/1591-951X-548.
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.
罗萨伊-多夫曼病(RDD)是1969年由罗萨伊和多夫曼描述的一种罕见的非朗格汉斯细胞组织细胞增生症。它是一种引人入胜的疾病,其特征是大的淡染组织细胞积聚,常表现为血细胞吞噬现象。罗萨伊教授深入研究了其多样的病理表现和不同临床形式的范围。尽管最近在RDD发病机制的剖析方面取得了进展,发现了丝裂原活化蛋白激酶(MAP)信号通路中的基因突变,但该疾病的一些生物学和临床方面仍有待阐明:这是罗萨伊教授留下的遗产之一。