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Related Experiment Videos

Leukocyte adhesion deficiency: clinical and postmortem observations.

H K Hawkins1, S C Heffelfinger, D C Anderson

  • 1Department of Pathology, Baylor College of Medicine, Houston, Texas.

Pediatric Pathology
|January 1, 1992
PubMed
Summary

Severe leukocyte adhesion deficiency (LAD) in an infant impaired neutrophil function, leading to recurrent infections and death. The study highlights CD18-independent neutrophil emigration and compromised lymphocyte function in LAD.

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Area of Science:

  • Immunology
  • Cell Biology
  • Pediatric Medicine

Background:

  • Leukocyte adhesion deficiency (LAD) is a rare primary immunodeficiency characterized by impaired neutrophil function.
  • Severe forms of LAD involve defects in adhesion molecules crucial for immune cell trafficking.

Observation:

  • An 18-month-old female presented with delayed umbilical cord separation, necrotizing skin lesions, and gingivitis.
  • Neutrophils lacked CD11/CD18 glycoproteins and exhibited deficient adhesion-dependent functions.
  • Autopsy revealed necrotizing enterocolitis, peritonitis, pneumonia, mucosal ulceration, and widespread microbial overgrowth without acute inflammation.

Findings:

  • Impaired neutrophil emigration from vasculature into tissues was the primary pathogenic mechanism.
  • Specific bronchopneumonia foci showed neutrophil infiltration, suggesting CD18-independent emigration pathways.

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  • Severe lymphocyte depletion was observed in lymphoid tissues, including the thymus.

    • Implications:

    • Neutrophil emigration can occur via CD18-independent mechanisms in response to certain stimuli.
    • Severe CD18 deficiency profoundly impacts lymphocyte function and immune surveillance in vivo.
    • This case underscores the critical role of neutrophil adhesion in host defense against microbial infections.