[Granulocyte disease caused by glycoprotein complex deficiency correlated with leukocyte adhesion].
This report summarizes the clinical and laboratory findings from a group of our patients and literature reviews from several families who have a genetic deficiency in three related leukocyte membrane surface antigens known as CR3,LFA-1, and p150,95. Each surface antigen has an identical beta-chain noncovalenty linked to one of three distinct alpha-chain types. Patients affected by this autosomal recessive disease have now been identified by several investigators. The patients have an increased susceptibility to bacterial infections that is similar to patients who have other types of neutrophil functional deficiencies or neutropenia. Laboratory tests have indicated that isolated neutrophils from these patients have two major types of functional deficiencies that probably contribute to their reduced ability to overcome bacterial infections. This review focuses exclusively on the phagocytic and cytotoxic abnormalities of neutrophils from these patients.
Ricevuti
G
G
Mazzone
A
A
Pasotti
D
D
Fioravanti
A
A
Uccelli
E
E
Notario
A
A
D000911:Antibodies, Monoclonal; D000943:Antigens, Differentiation / Q000172:deficiency*; D002448:Cell Adhesion; D006098:Granulocytes / Q000378:metabolism; D006801:Humans; D016167:Integrin alphaXbeta2; D018370:Leukocyte-Adhesion Deficiency Syndrome; D007962:Leukocytes / Q000378:metabolism*; D016169:Lymphocyte Function-Associated Antigen-1; D016177:Macrophage-1 Antigen; D011951:Receptors, Complement / Q000172:deficiency*
D000911:Antibodies, Monoclonal; D000943:Antigens, Differentiation; D016167:Integrin alphaXbeta2; D016169:Lymphocyte Function-Associated Antigen-1; D016177:Macrophage-1 Antigen; D011951:Receptors, Complement
La malattia dei granulociti da carenza del complesso glicoproteico correlato all'adesione leucocitaria.