Neutrophil adhesion in leukocyte adhesion deficiency syndrome type 2

J Clin Invest. 1995 Dec;96(6):2898-906. doi: 10.1172/JCI118361.


We have previously reported a newly discovered congenital disorder of neutrophil adhesion, leukocyte adhesion deficiency syndrome type 2 (LAD II). The clinical manifestations of this syndrome are similar to those seen in the classic leukocyte adhesion deficiency syndrome, now designated type 1 (LAD I), but the two syndromes differ in the molecular basis of their adhesion defects. LAD I is caused by a deficiency in the CD18 integrin adhesion molecules while LAD II patients are deficient in expression of sialyl-Lewis X (SLeX), a carbohydrate ligand for selectins. In this report we demonstrate that neutrophils from a LAD II patient bind minimally or not at all to recombinant E-selectin, purified platelet P-selectin, or P-selectin expressed on histamine-activated human umbilical vein endothelial cells, but have normal levels of L-selectin and CD11b/CD18 integrin, and adhere to and migrate across endothelium when CD11b/CD18 is activated. We compare LAD I and LAD II patient neutrophil function in vitro, demonstrating that integrin and selectin adhesion molecules have distinct but interdependent roles in neutrophil adhesion during an inflammatory response.

Publication types

  • Case Reports
  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • CD11 Antigens / physiology
  • CD18 Antigens / physiology
  • Cell Adhesion
  • Cells, Cultured
  • Chemotaxis, Leukocyte
  • E-Selectin / physiology
  • Endothelium, Vascular / physiology
  • Female
  • Flow Cytometry
  • Humans
  • In Vitro Techniques
  • Leukocyte-Adhesion Deficiency Syndrome / blood*
  • Leukocyte-Adhesion Deficiency Syndrome / classification
  • Male
  • Neutrophils / physiology*
  • P-Selectin / physiology
  • Probability
  • Reference Values
  • Umbilical Veins


  • CD11 Antigens
  • CD18 Antigens
  • E-Selectin
  • P-Selectin