Preclinical Evaluation for the Gene Therapy of Patients with Leukocyte Adhesion Deficiency Type I

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Leukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction

The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...

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Reversion Mutations in Patients with Leukocyte Adhesion Deficiency Type I (lad-i)

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Periodontal manifestation of leukocyte adhesion deficiency type I.

BACKGROUND Leukocyte adhesion deficiency type I (LAD-I) is an autosomal recessive immunodeficiency disorder characterized by defects in the integrin receptors of white blood cells that lead to impaired adhesion and chemotaxis. Affected patients are susceptible to recurrent bacterial and fungal infections, impaired pus formation, delayed wound healing, and periodontitis. METHODS A case of gene...

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Clinical profile of leukocyte adhesion deficiency type I.

Leukocyte adhesion deficiency type I (LAD-I) is a rare, inherited immunodeficiency with defect in the recruitment of leukocyte to the site of inflammation. Patients with severe LAD-I have absent or markedly reduced expression of CD18 and CD11. Here we report clinical profile of 7 cases of LAD-I diagnosed at our center over a period of 3 years. Recurrent skin and mucous membrane infections wer...

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ژورنال

عنوان ژورنال: Blood

سال: 2018

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood-2018-99-111136