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Absence of complement receptor type 3 and lymphocyte function antigen 1 causing deficient phagocyte and lymphocyte functions.

M M Manzionna·R A Seger·V Wahn·R M Zinkernagel·A Böcker·W H Hitzig
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Abstract

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.

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Antigens, DifferentiationBacterial InfectionsFemaleHumansImmune System DiseasesInfantKiller Cells, NaturalLymphocyte Function-Associated Antigen-1NeutrophilsReceptors, ComplementReceptors, Complement 3bRecurrenceCase ReportsJournal ArticleResearch Support, Non-U.S. Gov't

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M M Manzionna, R A Seger, V Wahn, R M Zinkernagel, A Böcker, W H Hitzig. 1988. Absence of complement receptor type 3 and lymphocyte function antigen 1 causing deficient phagocyte and lymphocyte functions.. https://doi.org/10.1007/bf00441816

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