Rosai-Dorfman disease presenting as mediastinal lymphadenopathy: case report and review of the literature. (1st May 2013)

• Record Type: Journal Article
• Authors: Cobanoglu, Nazan; Galip, Nilufer; Dalkan, Ceyhun; Comunoglu, Cem; Bahceciler, Nerin
• Is Part Of: Paediatrics and international child health. Volume 33:Number 2(2013:May)
• Page Start: 120
• Page End: 123
• Publication Date: 2013-05-01
• Subjects: Mediastinal lymphadenomegaly -- Rosai-Dorfman disease -- Immune deficiency -- Corticosteroids -- Intravenous immunoglobulin -- Emperipolesis; Pediatrics -- Developing countries -- Periodicals; Children -- Health and hygiene -- Developing countries -- Periodicals; Children -- Diseases -- Developing countries -- Periodicals; 618.920009172405
• DOI: 10.1179/2046905512Y.0000000025
• Languages: English
• ISSNs: 2046-9047

Abstract

Abstract : A 6-year-old boy presented with recurrent pneumonia and diarrhoea for 3 years. He had extensive mediastinal lymphadenopathy and atelectasis with low serum levels of IgA, IgG and IgG3. An inguinal lymph node biopsy demonstrated histological and histochemical features of Rosai-Dorfman disease. There was emperipolesis and histiocytes were immune-positive for S100 proteins. He responded to corticosteroids and regular infusions of immunoglobulins.