Leukocyte adhesion deficiency I

Leukocyte adhesion deficiency type I is caused by a mutation in the common chain of the β2 integrin family CD18. There are three β2 integrins with different α chains but a common β chain, called CD18. Defects in CD18 determine the loss of β2 integrin and the clinical symptoms. Neutrophils are unable to aggregate and do not bind to intercellular adhesion molecules on endhothelial cells. When there is no infection, the neutrophil count is about twice the normal level. LAD I has been described in more than 300 patients worldwide.

Alternative names


Lymphocyte function-associated antigen 1 immunodeficiency

LFA1 immunodeficiency

Leukocyte adhesion deficiency type 1, CD18 (integrin beta2)

Rambam-Hasharon syndrome


  • Defects of Phagocyte Function
    • Leukocyte adhesion defects


Autosomal recessive


#116920 Leukocyte adhesion deficiency, type I; LAD

*600065 Integrin, beta-2; ITGB2

Cross references

Phenotypically related immunodeficiencies

IDR factfile for Leukocyte adhesion deficiency II


Incidence is not known.