Rosai-Dorfman disease presenting as mediastinal lymphadenopathy: case report and review of the literature.
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.
Department of Paediatrics, Faculty of Medicine, Near East University, Nicosia, Cyprus. drncobanoglu@yahoo.com
Nazan
N
Cobanoglu
Nilufer
N
Galip
Ceyhun
C
Dalkan
Cem
C
Comunoglu
Nerin
N
Bahceciler
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D001706:Biopsy; D002648:Child; D015618:Histiocytosis, Sinus / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D006651:Histocytochemistry; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage; D007150:Immunohistochemistry; D007155:Immunologic Factors / Q000008:administration & dosage; D008206:Lymphatic Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D008297:Male; D009418:S100 Proteins / Q000032:analysis
D000305:Adrenal Cortex Hormones; D007136:Immunoglobulins; D007155:Immunologic Factors; D009418:S100 Proteins