Immunodeficiency in familial erythrophagocytic lymphohistiocytosis.
4 children with familial erythrophagocytic lymphohistiocytosis and hyperlipidaemia were found to have a previously unrecognised immunological deficiency syndrome which included defects in both humoral and cellular immunity and a plasma inhibitor of in-vitro lymphocyte blastogenesis. The inhibitory activity was proportional to the increase in the triglyceride concentration in the patients' plasma. Immunological deficiency, to which hyperlipidaemia may be a contributing factor, appears to be a significant feature of familial erythrophagocytic lymphohistiocytosis.
D000920:Antibody-Dependent Cell Cytotoxicity; D002455:Cell Division; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000276:immunology; D006644:Histiocytes / Q000276:immunology; D006801:Humans; D006949:Hyperlipidemias / Q000276:immunology; D007111:Immunity, Cellular; D007136:Immunoglobulins / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000276:immunology; D066298:In Vitro Techniques; D007223:Infant; D008206:Lymphatic Diseases / Q000235:genetics* / Q000276:immunology; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D010587:Phagocytosis; D014280:Triglycerides / Q000097:blood
D007136:Immunoglobulins; D014280:Triglycerides