Altered erythrocyte endothelial adherence and membrane phospholipid asymmetry in hereditary hydrocytosis. Blood 2003 Jun 01;101(11):4625-7
Date
02/01/2003Pubmed ID
12560240DOI
10.1182/blood-2001-12-0329Scopus ID
2-s2.0-0038481238 (requires institutional sign-in at Scopus site) 218 CitationsAbstract
The risk for thrombosis is increased in patients with hereditary hydrocytosis, an uncommon variant of hereditary stomatocytosis. Erythrocytes from 2 patients with hydrocytosis were studied to gain insight into the mechanism of thrombosis in this disorder. Erythrocytes demonstrated abnormal osmotic scan ektacytometry and decreased erythrocyte filtration rates. There was also a mild increase in adherence of erythrocytes to endothelial monolayers in a micropipette assay. Adhesion of erythrocytes to the subendothelial matrix proteins thrombospondin and laminin, however, was not significantly increased. Percentages of hydrocytosis erythrocytes and reticulocytes with phosphatidylserine exposed on the outer surfaces were increased in both patients compared with healthy controls, indicating altered membrane phospholipid asymmetry. Increased phosphatidylserine exposure accelerating thrombin-forming processes has been proposed as a mechanism for thrombosis in sickle cell disease and beta-thalassemia and may play a similar role in hereditary hydrocytosis.
Author List
Gallagher PG, Chang SH, Rettig MP, Neely JE, Hillery CA, Smith BD, Low PSMESH terms used to index this publication - Major topics in bold
AdultAnemia, Hemolytic, Congenital
Case-Control Studies
Cell Adhesion
Child
Endothelium, Vascular
Erythrocyte Membrane
Erythrocytes
Family Health
Humans
Male
Phosphatidylserines
Thrombosis
Umbilical Veins
