leukocyte adhesion deficiency-1

Pathology

• impaired phagocyte adherence, aggregation, spreading, chemotaxis, phagocytosis of C3bi-coated particles
• defective production of CD18 subunit common to leukocyte integrins

Genetics

autosomal recessive

Clinical manifestations

• delayed separation of umbilical cord
• recurrent infections of skin & mucosa
• gingivitis
• periodontal disease

Laboratory

• sustained granulocytosis
• decreased phagocyte expression of CD18-containing integrins using monoclonal antibodies
  • LFA-1 Mac-1, CD18/CD11c
• see ARUP consult

More general terms

• leukocyte adhesion deficiency

Additional terms

• CD18 (integrin beta-2, L-CAM beta, ITGB2, CD18, MFI7)
• complement C3b-inactivated
• integrin