Rosai-Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report.
A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome) in a 42-year-old man is described. The disease involved the respiratory tract and skeletal apparatus, led to considerable general impairment and was unusually responsive to glucocorticoids. Molecular and immunophenotype analysis seem to confirm the reactive nature of a disorder that shows profound T-cell immunodeficiency.
Sita
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G
Servizio Autonomo di Ematologia Diagnostica, Centro G. Marcora, Ospedale Maggiore IRCCS, Università degli Studi, Milano, Italy.
Guffanti
A
A
Colombi
M
M
Ferrari
A
A
Neri
A
A
Baldini
L
L
D000328:Adult; D001847:Bone Diseases / Q000188:drug therapy* / Q000209:etiology; D005938:Glucocorticoids / Q000627:therapeutic use*; D015618:Histiocytosis, Sinus / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D012140:Respiratory Tract Diseases / Q000188:drug therapy* / Q000209:etiology
D005938:Glucocorticoids