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History of Rosai-Dorfman Disease

In 1969, Dr. Juan Rosai and Dr. Ronald F. Dorfman reported four cases of an entity that previously had puzzled pathologists and clinicians. The four cases had failed to fit under any diagnosis, but shared a few common characteristics. Each patient presented with painless, massive lymphadenopathy, most commonly of the cervical lymph nodes1. Other locations included the inguinal, intra-parotid, and axillary lymph nodes. The patients all presented with fever and leukocytosis1. The differential diagnosis included malignant lymphoma, malignant histiocytosis, reticuloendotheliosis, and chronic inflammation. However, the histopathological characteristics of the cases did not fit the classical characteristics of these diagnoses. Based on the pathologic findings, Dr. Rosai and Dr. Dorfman created a new entity, which they called “sinus histiocytosis with massive lymphadenopathy (SHML).”1

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