title: Immunological studies on histiocytosis X. I. Special reference to the chemotactic defect and the HLA antigen.
abstract: We treated a family with three children with histiocytosis X (H-X). The chemotactic response of the neutrophils in these three patients was depressed and the chemotactic response of the neutrophils of the mother was also depressed compared to that of normal age-matched controls. To elucidate the genetic factors, we examined HLA antigens in five members of this family. All five members had Aw24, B7, Cw7, and DR1. Immunological and genetic studies in an additional 32 patients with H-X were performed. The chemotactic response of 35 patients with H-X (154.9 +/- 58.4/HPF) was significantly depressed in comparison with that of 35 age-matched healthy controls (613.3 +/- 116.7/HPF). In addition, the value of chemiluminescence of 20 of 35 patients (20.5 +/- 6.6 mV) was also significantly depressed in comparison with that of 20 normal controls (45.3 +/- 11.4 mV). The frequencies of Bw61 (54.4%) and Cw7 (45.4%) in 33 patients with H-X were significantly increased in comparison with those of 250 normal healthy controls (20.4 and 18.0%, respectively). Studies of immunoglobulin levels and complement titers of patients with H-X showed no consistent abnormalities. We proposed that defects of polymorphonuclear function may lead to an increased susceptibility to bacterial infections in patients with this disorder.
mesh_terms: D000293:Adolescent; D001770:Blood Bactericidal Activity; D002630:Chemotactic Factors / Q000172:deficiency*; D002634:Chemotaxis, Leukocyte; D002648:Child; D002675:Child, Preschool; D003165:Complement System Proteins / Q000032:analysis; D005260:Female; D006680:HLA Antigens / Q000032:analysis* / Q000276:immunology; D006646:Histiocytosis, Langerhans-Cell / Q000097:blood / Q000235:genetics / Q000276:immunology*; D006650:Histocompatibility Testing; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007223:Infant; D008297:Male; D009504:Neutrophils
chemical_list: D002630:Chemotactic Factors; D006680:HLA Antigens; D007136:Immunoglobulins; D003165:Complement System Proteins