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[Granulocyte disease caused by glycoprotein complex deficiency correlated with leukocyte adhesion]
Insights
Patients with a genetic deficiency in CR3, LFA-1, and p150,95 leukocyte antigens exhibit increased bacterial infections due to neutrophil functional abnormalities.
Area of Science:
- Immunology
- Genetics
- Cell Biology
Context:
- Focuses on leukocyte adhesion molecule deficiencies.
- Reviews clinical and laboratory findings in affected families.
- Highlights the role of CR3, LFA-1, and p150,95 antigens.
Purpose:
- To summarize clinical and laboratory findings of patients with genetic deficiencies in CR3, LFA-1, and p150,95.
- To review the neutrophil functional abnormalities in these patients.
- To correlate these deficiencies with increased susceptibility to bacterial infections.
Summary:
- Describes an autosomal recessive disease affecting leukocyte membrane surface antigens (CR3, LFA-1, p150,95).
- Each antigen comprises a common beta-chain and distinct alpha-chains.
- Patients present with neutrophil functional deficiencies, impacting phagocytosis and cytotoxicity.
- This leads to increased susceptibility to bacterial infections, similar to other neutrophil disorders.
Impact:
- Enhances understanding of leukocyte adhesion molecule function in immunity.
- Provides insights into neutrophil dysfunction and its clinical consequences.
- Contributes to the diagnosis and management of primary immunodeficiencies.
Abstract:
This report summarizes the clinical and laboratory findings from a group of our patients and literature reviews from several families who have a genetic deficiency in three related leukocyte membrane surface antigens known as CR3,LFA-1, and p150,95. Each surface antigen has an identical beta-chain noncovalenty linked to one of three distinct alpha-chain types. Patients affected by this autosomal recessive disease have now been identified by several investigators. The patients have an increased susceptibility to bacterial infections that is similar to patients who have other types of neutrophil functional deficiencies or neutropenia. Laboratory tests have indicated that isolated neutrophils from these patients have two major types of functional deficiencies that probably contribute to their reduced ability to overcome bacterial infections. This review focuses exclusively on the phagocytic and cytotoxic abnormalities of neutrophils from these patients.