Extranodal Rosai-Dorfman disease in childhood

Pathology, Research and Practice
S RemadiT A Seemayer

Abstract

We describe two children with Rosai-Dorfman disease who presented with exophthalmos, leukocytosis, an elevated sedimentation rate and hypergammaglobulinemia. Both became blind as a result of this condition. One child had associated bilateral cervical lymphadenopathy. Investigation revealed involvement of the nasal fossae and retro-orbital spaces by tumoural masses histologically consistent with the diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML). Immunohistochemical studies suggest that these histiocytes are activated macrophages derived from a phenotype hybrid between "professional" phagocytic cells and immune accessory cells, expressing lysosomal antigens, S-100 protein but rarely, CDla. An underlying immune dysfunction may be central to the pathogenesis of this disease.

References

May 1, 1979·AJR. American Journal of Roentgenology·M J SiegelW H McAlister
Feb 1, 1979·The Journal of the Medical Society of New Jersey·K SaranathanE Wagman
Jun 1, 1992·Leukemia & Lymphoma·S SacchiG Emilia
Jan 1, 1992·The Journal of Laryngology and Otology·R G Courteney-Harris, M J Goddard
Feb 1, 1992·The American Journal of Surgical Pathology·E A MontgomeryG Frizzera
Jan 1, 1991·Virchows Archiv. A, Pathological Anatomy and Histopathology·F DelacrétazJ P Chave
Feb 1, 1994·The Journal of Laryngology and Otology·R T Gregor, D Ninnin

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Citations

Jul 1, 2008·Journal of Hematology & Oncology·Geeta K VemugantiSantosh G Honavar
May 22, 2002·Ophthalmic Plastic and Reconstructive Surgery·Chee-Chew YipChai-Teck Choo
May 23, 2006·Ophthalmic Plastic and Reconstructive Surgery·Yasaman MohadjerPhilip L Custer
Oct 31, 2008·Journal français d'ophtalmologie·V PaireA Péchereau

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