TY - JOUR
T1 - Leukocyte adhesion deficiencies
AU - van de Vijver, Edith
AU - van den Berg, Timo K
AU - Kuijpers, Taco W
N1 - Copyright © 2013 Elsevier Inc. All rights reserved.
PY - 2013/2
Y1 - 2013/2
N2 - During inflammation, leukocytes play a key role in maintaining tissue homeostasis through elimination of pathogens and removal of damaged tissue. Leukocytes migrate to the site of inflammation by crawling over and through the blood vessel wall, into the tissue. Leukocyte adhesion deficiencies (ie, LAD-I, -II, and LAD-I/variant, the latter also known as LAD-III) are caused by defects in the adhesion of leukocytes to the vessel wall, resulting in severe recurrent nonpussing infections and neutrophilia, often preceded by delayed separation of the umbilical cord. Although dependent on the genetic defect, hematopoietic stem cell transplantation is often the only curative treatment.
AB - During inflammation, leukocytes play a key role in maintaining tissue homeostasis through elimination of pathogens and removal of damaged tissue. Leukocytes migrate to the site of inflammation by crawling over and through the blood vessel wall, into the tissue. Leukocyte adhesion deficiencies (ie, LAD-I, -II, and LAD-I/variant, the latter also known as LAD-III) are caused by defects in the adhesion of leukocytes to the vessel wall, resulting in severe recurrent nonpussing infections and neutrophilia, often preceded by delayed separation of the umbilical cord. Although dependent on the genetic defect, hematopoietic stem cell transplantation is often the only curative treatment.
KW - Congenital Disorders of Glycosylation
KW - Humans
KW - Leukocyte-Adhesion Deficiency Syndrome/complications
KW - Prognosis
U2 - 10.1016/j.hoc.2012.10.001
DO - 10.1016/j.hoc.2012.10.001
M3 - Review article
C2 - 23351991
VL - 27
SP - 101-16, viii
JO - Hematology/Oncology Clinics of North America
JF - Hematology/Oncology Clinics of North America
SN - 0889-8588
IS - 1
ER -