Extranodal Rosai-Dorfman Disease in Childhood

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 involveme...

Full description

Saved in:
Bibliographic Details
Published in:Pathology, research and practice research and practice, 1996-10, Vol.192 (10), p.1007-1015
Main Authors: Remadi, S., Doussis Anagnostopoulou, I., Jlidi, R., Cox, J.N., Seernayer, T.A.
Format: Article
Language:English
Subjects:
Antigens, CD1 - analysis
Child
Extranodal Rosai-Dorfman disease
Histiocytosis, Sinus - immunology
Histiocytosis, Sinus - pathology
Humans
Immunohistochemistry
Lymph Nodes - pathology
Male
Nasal Cavity - immunology
Nasal Cavity - pathology
Orbit - immunology
Orbit - pathology
S100 Proteins - analysis
Sinus histiocytosis with massive lymphadenopathy
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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, CD1a. An underlying immune dysfunction may be central to the pathogenesis of this disease.
ISSN:0344-0338
1618-0631
DOI:10.1016/S0344-0338(96)80042-2