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.
MeSH terms
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Adrenal Cortex Hormones / administration & dosage
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Biopsy
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Child
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Histiocytosis, Sinus / diagnosis*
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Histiocytosis, Sinus / drug therapy
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Histiocytosis, Sinus / pathology*
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Histocytochemistry
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Humans
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Immunoglobulins / administration & dosage
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Immunohistochemistry
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Immunologic Factors / administration & dosage
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Lymphatic Diseases / drug therapy
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Lymphatic Diseases / etiology*
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Lymphatic Diseases / pathology*
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Male
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S100 Proteins / analysis
Substances
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Adrenal Cortex Hormones
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Immunoglobulins
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Immunologic Factors
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S100 Proteins