ROSAI-DORFMAN SYNDROME WITH EXTRANODAL LOCALIZATIONS AND RESPONSE TO GLUCOCORTICOIDS - A CASE-REPORT

Authors
SITA G GUFFANTI A COLOMBI M FERRARI A NERI A BALDINI L
Citation
G. Sita et al., ROSAI-DORFMAN SYNDROME WITH EXTRANODAL LOCALIZATIONS AND RESPONSE TO GLUCOCORTICOIDS - A CASE-REPORT, Haematologica, 81(2), 1996, pp. 165-167
Citations number
12
Categorie Soggetti
Hematology
Journal title
Haematologica → ACNP
ISSN journal
03906078
Volume
81
Issue
2
Year of publication
1996
Pages
165 - 167
Database
ISI
SICI code
0390-6078(1996)81:2<165:RSWELA>2.0.ZU;2-S
Abstract
A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorf man syndrome) in a 42-year-old man is described. The disease involved the respiratory tract and skeletal apparatus, led to considerable gene ral impairment and was unusually responsive to glucocorticoids. Molecu lar and immunophenotype analysis seem to confirm the reactive nature o f a disorder that shows profound T-cell immunodeficiency.