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以纵隔淋巴结肿大为表现的罗萨伊-多夫曼病:病例报告及文献复习

Rosai-Dorfman disease presenting as mediastinal lymphadenopathy: case report and review of the literature.

作者信息

Cobanoglu Nazan, Galip Nilufer, Dalkan Ceyhun, Comunoglu Cem, Bahceciler Nerin

机构信息

Department of Paediatrics, Faculty of Medicine, Near East University, Nicosia, Cyprus.

出版信息

Paediatr Int Child Health. 2013 May;33(2):120-3. doi: 10.1179/2046905512Y.0000000025.

DOI:10.1179/2046905512Y.0000000025
PMID:23925289
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.

摘要

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。

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