Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Rosai-Dorfman Disease, Pleural

  • Daniel GehlbachEmail author
Reference work entry


Destombes-Rosai-Dorfman; DRDD; RDD; SHML; Sinus histiocytosis with massive lymphadenopathy


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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References and Further Reading

  1. Filipovich, M. B. J. a. A. H. (2012). Hoffman: hematology: Basic principles and practice (6th ed.). 699–700.Google Scholar
  2. 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.PubMedGoogle Scholar
  3. 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.CrossRefPubMedGoogle Scholar
  4. Ohori, N. P., et al. (2003). Rosai-Dorfman disease of the pleura: A rare extranodal presentation. Human Pathology, 34(11), 1210–1211.CrossRefPubMedGoogle Scholar
  5. Rosai, J., & Dorfman, R. F. (1969). Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Archives of Pathology, 87(1), 63–70.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.UT Southwestern Medical Center/Parkland Hospital and Health SystemDallasUSA