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- W2074927145 abstract "Omenn's syndrome is a rare, usually fatal immunologic disorder of infancy characterized by recurrent infections, skin lesion, lymphadenopathy, peripheral blood lymphocytosis, and eosinophilia. Histologic evaluation of a lymph node revealed total effacement of the microscopic architecture resulting from a diffuse proliferation of interdigitating reticulum cells and a depletion of B lymphocytes. The lymph node lacked a distinct cortex and had no follicle formation. The most striking feature was a diffuse hyperplasia of S-100-protein-positive nonphagocytic reticulum cells with large, pale Langerhans-like nuclei. Ultrastructural examination identified these cells to be interdigitating reticulum cells. The lymphocytes were small and predominantly of the CD8 cytotoxic/suppressor cell type." @default.
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- W2074927145 date "1995-09-01" @default.
- W2074927145 modified "2023-10-18" @default.
- W2074927145 title "The Lymph Node Pathology of Omennʼs Syndrome" @default.
- W2074927145 doi "https://doi.org/10.1097/00000478-199509000-00013" @default.
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