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Clinical and Osteopetrosis-Like Radiological Findings in Patients with Leukocyte Adhesion Deficiency Type III.

Ayça Burcu KahramanIsmail YazRahsan GocmenSelin AytacAyse MetinSara Sebnem KilicIlhan TezcanDeniz Cagdas
Published in: Journal of clinical immunology (2023)
In LAD-III, leukocytosis, eosinophilia, and bone marrow findings may mimic pathologies such as JMML and MDS. In addition to non-purulent infection susceptibility, patients with LAD-III exhibit Glanzmann-type bleeding disorder. In LAD-III, absent integrin activation due to kindlin-3 deficiency disrupts osteoclast actin cytoskeleton organization. This results in defective bone resorption and osteopetrosis-like radiological changes. These are distinctive features compared to other LAD types.
Keyphrases
  • type iii
  • bone marrow
  • bone loss
  • cell migration
  • mesenchymal stem cells
  • bone mineral density
  • atrial fibrillation
  • replacement therapy
  • smoking cessation
  • peripheral blood
  • soft tissue
  • cystic fibrosis