Document Detail

Loss of endothelial surface expression of E-selectin in a patient with recurrent infections.
MedLine Citation:
PMID:  10419878     Owner:  NLM     Status:  MEDLINE    
Neutrophil accumulation at sites of inflammation is mediated by specific groups of cell adhesion molecules including the beta2 (CD18) integrins on leukocytes and the selectins (P- and E-selectin on the endothelium and L-selectin on the leukocyte). This is supported by studies of patients with leukocyte adhesion deficiency syndromes whose leukocytes are genetically deficient in the expression of beta2 integrins or selectin carbohydrate ligands (eg, sialyl-Lewis(x)). However, inherited deficiency or dysfunction of endothelial cell adhesion molecules involved in leukocyte recruitment has not been previously described. In this report we describe a child with recurrent infections and clinical evidence of impaired pus formation reminiscent of a leukocyte adhesion deficiency syndrome, but whose neutrophils were functionally normal and expressed normal levels of CD18, L-selectin, and sialyl-Lewis(x). In contrast, immunohistochemical staining of inflamed tissue from the patient showed the absence of E-selectin from the endothelium, although E-selectin mRNA was present. However, E-selectin protein was expressed as significantly elevated levels of circulating soluble E-selectin were detected, the molecular size of which was consistent with a proteolytically cleaved form of E-selectin. Gene sequencing failed to show evidence of a secreted mutant variant. These data represent, to our knowledge, the first description of a potentially inherited dysfunction of an endothelial cell adhesion molecule involved in leukocyte recruitment and provide additional human evidence of the importance of endothelial selectins in the inflammatory response.
H M DeLisser; M Christofidou-Solomidou; J Sun; M T Nakada; K E Sullivan
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Blood     Volume:  94     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  1999 Aug 
Date Detail:
Created Date:  1999-08-17     Completed Date:  1999-08-17     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  884-94     Citation Subset:  AIM; IM    
Pulmonary and Critical Care Division, University of Pennsylvania Medical Center, Philadelphia, PA, USA.
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MeSH Terms
Bacterial Infections / immunology*,  pathology
Cell Adhesion
Cell Movement / immunology
E-Selectin / biosynthesis*,  genetics
Endothelium, Vascular / immunology*,  metabolism,  pathology
Immunity, Innate / genetics
Inflammation / genetics,  immunology
Leukocytes / immunology,  pathology
Grant Support
Reg. No./Substance:
Comment In:
Blood. 1999 Dec 1;94(11):3956   [PMID:  10627121 ]

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