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
Bloodjournal_title
Bloodauthors
Gallagher PG,Chang SH,Rettig MP,Neely JE,Hillery CA,Smith BD,Low PSdoi
10.1182/blood-2001-12-0329subject
Has Abstractpub_date
2003-06-01 00:00:00pages
4625-7issue
11eissn
0006-4971issn
1528-0020pii
2001-12-0329journal_volume
101pub_type
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