Altered erythrocyte endothelial adherence and membrane phospholipid asymmetry in hereditary hydrocytosis

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 s...

Full description

Saved in:
Bibliographic Details
Published in:Blood 2003-06, Vol.101 (11), p.4625-4627
Main Authors: Gallagher, Patrick G., Chang, Seon Hee, Rettig, Michael P., Neely, John E., Hillery, Cheryl A., Smith, Brian D., Low, Philip S.
Format: Article
Language:English
Subjects:
Adult
Anemia, Hemolytic, Congenital - etiology
Anemia, Hemolytic, Congenital - pathology
Anemias. Hemoglobinopathies
Biological and medical sciences
Blood and lymphatic vessels
Cardiology. Vascular system
Case-Control Studies
Cell Adhesion
Child
Diseases of red blood cells
Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous
Endothelium, Vascular - cytology
Erythrocyte Membrane - physiology
Erythrocyte Membrane - ultrastructure
Erythrocytes - pathology
Family Health
Hematologic and hematopoietic diseases
Humans
Male
Medical sciences
Phosphatidylserines
Thrombosis - etiology
Umbilical Veins - cytology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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 β-thalassemia and may play a similar role in hereditary hydrocytosis.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2001-12-0329