Cobanoglu Nazan, Galip Nilufer, Dalkan Ceyhun, Comunoglu Cem, Bahceciler Nerin
Department of Paediatrics, Faculty of Medicine, Near East University, Nicosia, Cyprus.
Paediatr Int Child Health. 2013 May;33(2):120-3. doi: 10.1179/2046905512Y.0000000025.
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.
一名6岁男孩出现反复肺炎和腹泻3年。他有广泛的纵隔淋巴结肿大和肺不张,血清IgA、IgG和IgG3水平低。腹股沟淋巴结活检显示了罗萨伊-多夫曼病的组织学和组织化学特征。存在血细胞吞噬现象,组织细胞对S100蛋白免疫阳性。他对皮质类固醇和定期输注免疫球蛋白有反应。