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leukocyte adhesion deficiency-1
Genetics: autosomal recessive
Pathology:
1) impaired phagocyte adherence, aggregation, spreading, chemotaxis, phagocytosis of C3bi-coated particles
2) defective production of CD18 subunit common to leukocyte integrins
Clinical manifestations:
1) delayed separation of umbilical cord
2) recurrent infections of skin & mucosa
3) gingivitis
4) periodontal disease
Laboratory:
1) sustained granulocytosis
2) decreased phagocyte expression of CD18-containing integrins using monoclonal antibodies
-> LFA-1 Mac-1, CD18/CD11c
3) see ARUP consult [2]
Related
CD18 (integrin beta-2, L-CAM beta, ITGB2, CD18, MFI7)
complement C3b-inactivated
integrin
General
leukocyte adhesion deficiency
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 356
- ARUP Consult: Leukocyte Adhesion Deficiency
The Physician's Guide to Laboratory Test Selection & Interpretation
https://www.arupconsult.com/content/leukocyte-adhesion-deficiency