Rosai-Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report.
نویسندگان
چکیده
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.
منابع مشابه
Rosai-Dorfman Disease: A Case Report and Literature Review
Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disorder that typically manifests as lymphadenopathy and systemic symptoms whose etiology remains poorly elucidated. The diagnosis is based on immunohistochemistry. Its treatment is poorly defined but the prognosis is usually favorable. Here we report a 14 year old boy who presented with massive bilateral cervi...
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عنوان ژورنال:
- Haematologica
دوره 81 2 شماره
صفحات -
تاریخ انتشار 1996