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Defective neutrophil and lymphocyte function in leucocyte adhesion deficiency
1Department of Paediatrics, University of Hong Kong, Queen Mary Hospital.
Clinical and Experimental Immunology
|August 1, 1991
Summary
Leukocyte adhesion deficiency (LAD) impairs neutrophil function and cellular immunity in a Chinese girl. This case highlights defects in B cell, T cell, and natural killer cell activity, impacting immune responses.
Area of Science:
- Immunology
- Genetics
Background:
- Leukocyte adhesion deficiency (LAD) is a group of rare primary immunodeficiencies.
- It is characterized by defects in leukocyte adhesion and migration, leading to recurrent infections.
Observation:
- A Chinese girl presented with moderate LAD phenotype, including persistent omphalitis and recurrent soft tissue infections.
- She exhibited subnormal neutrophil functions, impaired B cell function, and defective T cell-dependent antibody production.
- Marked defects in natural killer cell activity were also observed.
Findings:
- The patient displayed impaired chemotaxis and chemiluminescence of neutrophils.
- In vitro studies showed reduced B cell function and impaired generation of plaque-forming cells (PFC), particularly IgG and IgA PFC.
- Helper function of CD4+ cells was virtually absent in pokeweed mitogen (PWM)-driven PFC assays, and natural killer cell activity was markedly defective.
Implications:
- This case underscores the complex immune dysregulation in LAD, affecting both innate and adaptive immunity.
- Understanding these defects is crucial for diagnosing and managing LAD patients.
- Further research into LAD pathogenesis can inform therapeutic strategies for primary immunodeficiencies.