Adult severe combined immunodeficiency and sarcoid-like granulomas with hypersplenism.
A patient presented with lymphopenia, anergy, hypogammaglobulinemia and hypersplenism. Histologic examination of the spleen and lymph node revealed noncaseating sarcoid-like granulomas. Despite a significant rise in circulating lymphocytes after splenectomy there was in vivo and in vitro evidence of B- and T-lymphocyte dysfunction. A histologic picture mimicking sarcoidosis may occur in patients with immune deficiency. The granulomatous proliferation may represent an altered host response to antigen.
D000328:Adult; D006099:Granuloma / Q000150:complications*; D006801:Humans; D006971:Hypersplenism / Q000150:complications*; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008198:Lymph Nodes / Q000473:pathology; D008213:Lymphocyte Activation; D008263:Macrophage Migration-Inhibitory Factors / Q000276:immunology; D008264:Macrophages / Q000276:immunology; D008297:Male; D008934:Mitogens / Q000494:pharmacology; D013154:Spleen / Q000473:pathology; D013299:Streptodornase and Streptokinase / Q000276:immunology
D008263:Macrophage Migration-Inhibitory Factors; D008934:Mitogens; D013299:Streptodornase and Streptokinase