Rosai-Dorfman Disease

Synonyms

Rosai-Dorfman-Destombes disease (RDD); Sinus histiocytosis with massive lymphadenopathy

Definition

Rosai-Dorfman disease (RDD) is a rare nodal or extranodal disorder defined by an accumulation of atypical histiocytes exhibiting emperipolesis, admixed with other hematopoietic elements, particularly, plasma cells and lymphocytes. The disease was established as a distinct clinical entity by Juan Rosai and Ronald Dorfman in two publications in 1969 and 1972 (Rosai and Dorfman 1969, 1972). Although “sinus histiocytosis with massive lymphadenopathy” (SHML) was the original name proposed by Rosai and Dorfman, the eponym “Rosai-Dorfman disease” (RDD) was later used to refer to the extranodal manifestations of this disease. Both names are currently used interchangeably. Rosai and Dorfman also established an SHML registry, which allowed them to collect and study 423 cases and their clinicopathologic characteristics in 1990 (Foucar et al. 1990). More recently, RDD has been shown to be...