Altered erythrocyte endothelial adherence and membrane phospholipid asymmetry in hereditary hydrocytosis.

Abstract:

: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.

journal_name

Blood

journal_title

Blood

authors

Gallagher PG,Chang SH,Rettig MP,Neely JE,Hillery CA,Smith BD,Low PS

doi

10.1182/blood-2001-12-0329

subject

Has Abstract

pub_date

2003-06-01 00:00:00

pages

4625-7

issue

11

eissn

0006-4971

issn

1528-0020

pii

2001-12-0329

journal_volume

101

pub_type

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