Synonyms
Destombes-Rosai-Dorfman; DRDD; RDD; SHML; Sinus histiocytosis with massive lymphadenopathy
Definition
Rosai-Dorfman disease (RDD) is a polyclonal, benign proliferation of macrophages with a histologically reactive morphology causing painless, chronic, massive lymphadenopathy and occasional extranodal manifestations. In 1992, a landmark study of RDD reported detection of HHV-6 genome by in situ hybridization in 7 out of 9 RDD patients evaluated and EBV genome in a single patient (Levine et al. 1992). Viral infections, namely, HHV-6, may be the underlying cause of RDD.
Clinical Features
Incidence
Rare; data from a disease registry of 423 patients is available from a 1990 report (Foucar et al. 1990).
Age
Patients are typically young with a mean age of onset at 20.6 years, but there may be wide age distribution at presentation (Rosai and Dorfman 1969; Foucar et al. 1990).
Sex
May be slightly more common in male patients overall (58% of patients); however, 6 of 9 patients with...
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Filipovich, M. B. J. a. A. H. (2012). Hoffman: hematology: Basic principles and practice (6th ed.). 699–700.
Foucar, E., et al. (1990). Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Seminars in Diagnostic Pathology, 7(1), 19–73.
Levine, P. H., et al. (1992). Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Journal of Infectious Diseases, 166(2), 291–295.
Ohori, N. P., et al. (2003). Rosai-Dorfman disease of the pleura: A rare extranodal presentation. Human Pathology, 34(11), 1210–1211.
Rosai, J., & Dorfman, R. F. (1969). Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Archives of Pathology, 87(1), 63–70.
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Gehlbach, D. (2018). Rosai-Dorfman Disease, Pleural. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_386
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DOI: https://doi.org/10.1007/978-3-319-66796-6_386
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