Understanding the Role of LFA-1 in Leukocyte Adhesion Deficiency Type I (LAD I): Moving towards Inflammation?

Int J Mol Sci. 2022 Mar 25;23(7):3578. doi: 10.3390/ijms23073578.


LFA-1 (Lymphocyte function-associated antigen-1) is a heterodimeric integrin (CD11a/CD18) present on the surface of all leukocytes; it is essential for leukocyte recruitment to the site of tissue inflammation, but also for other immunological processes such as T cell activation and formation of the immunological synapse. Absent or dysfunctional expression of LFA-1, caused by mutations in the ITGB2 (integrin subunit beta 2) gene, results in a rare immunodeficiency syndrome known as Leukocyte adhesion deficiency type I (LAD I). Patients suffering from severe LAD I present with recurrent infections of the skin and mucosa, as well as inflammatory symptoms complicating the clinical course of the disease before and after allogeneic hematopoietic stem cell transplantation (alloHSCT); alloHSCT is currently the only established curative treatment option. With this review, we aim to provide an overview of the intrinsic role of inflammation in LAD I.

Keywords: hematopoietic stem cell transplantation; inborn errors of immunity; inflammation.

Publication types

  • Review

MeSH terms

  • Cell Adhesion / genetics
  • Humans
  • Inflammation / genetics
  • Leukocyte-Adhesion Deficiency Syndrome* / genetics
  • Leukocyte-Adhesion Deficiency Syndrome* / therapy
  • Leukocytes
  • Lymphocyte Function-Associated Antigen-1* / genetics


  • Lymphocyte Function-Associated Antigen-1

Supplementary concepts

  • Leukocyte adhesion deficiency type 1