Absence of complement receptor type 3 and lymphocyte function antigen 1 causing deficient phagocyte and lymphocyte functions.
We describe a patient with delayed umbilical cord detachment, recurrent bacterial infections, and inability to form pus, despite persistent leucocytosis. Immunofluorescence studies with specific monoclonal antibodies showed a severe deficiency in the expression of alpha-chains of the receptor for the C3bi fragment of C3, complement receptor type 3, and the lymphocyte function antigen 1 molecule, found on neutrophil, monocyte and lymphocyte membranes. These membrane antigen defects were responsible for abnormalities in adhesive cell functions. Polymorphonuclear leucocytes demonstrated a markedly reduced chemiluminescence response as well as an impaired nitroblue tetrazolium test and superoxide generation to a particulate stimulus (zymosan), while the responses to a soluble stimulus (phorbol myristate acetate) were normal. In addition, random migration und chemotactic response to zymosan-activated serum were impaired. The lymphocytes demonstrated abolished natural killer cell cytotoxicity as well as abnormal humoral immunity and a lack of antibody response to pertussis and tetanus antigens.
Manzionna
M M
MM
Department of Paediatrics, Division of Immunology, Zürich, Switzerland.
Seger
R A
RA
Wahn
V
V
Zinkernagel
R M
RM
Böcker
A
A
Hitzig
W H
WH
D000943:Antigens, Differentiation / Q000276:immunology*; D001424:Bacterial Infections / Q000276:immunology; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000276:immunology*; D007223:Infant; D007694:Killer Cells, Natural / Q000276:immunology; D016169:Lymphocyte Function-Associated Antigen-1; D009504:Neutrophils / Q000276:immunology; D011951:Receptors, Complement / Q000276:immunology*; D017463:Receptors, Complement 3b; D012008:Recurrence
D000943:Antigens, Differentiation; D016169:Lymphocyte Function-Associated Antigen-1; D011951:Receptors, Complement; D017463:Receptors, Complement 3b