Defective neutrophil and lymphocyte function in leucocyte adhesion deficiency.
We report a Chinese girl with the moderate phenotype of leucocyte adhesion deficiency (LAD), presenting with persistent omphalitis and recurrent soft tissue infections. She had subnormal adhesion-dependent neutrophil functions, such as chemotaxis and chemiluminescence response to a particulate stimulant (opsonised zymosan). Despite her adequate humoral response to documented herpes simplex virus type 1, parainfluenza type 2 and adenovirus infection in vivo, there was marked impairment in the generation of plaque-forming cells (PFC) driven by pokeweed mitogen (PWM) in vitro. IgM PFC were less severely affected than IgG and IgA PFC, probably because IgM production is less dependent on T cell help than IgA and IgG production. The patient's B cells and accessory cells had reduced function compared with the control subsets, while helper function of her CD4+ cells was virtually absent in the PWM-driven PFC assay. She also had marked defect in natural killer cell activity. The proliferation of her lymphocytes was normal to several plant lectins, including phytohaemagglutinin, concanavalin A and PWM, but markedly defective to OKT3.
Department of Paediatrics, University of Hong Kong, Queen Mary Hospital.
Y L
YL
Lau
L C
LC
Low
B M
BM
Jones
J W
JW
Lawton
D015703:Antigens, CD / Q000172:deficiency; D018821:CD18 Antigens; D002448:Cell Adhesion; D002675:Child, Preschool; D005260:Female; D005434:Flow Cytometry; D005455:Fluorescent Antibody Technique; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology; D018370:Leukocyte-Adhesion Deficiency Syndrome; D008214:Lymphocytes / Q000276:immunology*; D016177:Macrophage-1 Antigen / Q000172:deficiency; D009504:Neutrophils / Q000276:immunology*
D015703:Antigens, CD; D018821:CD18 Antigens; D016177:Macrophage-1 Antigen