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.

• Authors: Patrick G Gallagher, Seon Hee Chang, Michael P Rettig, John E Neely, Cheryl A Hillery, Brian D Smith, Philip S Low
• Journal: Blood (Blood) Vol. 101 Issue 11 Pg. 4625-7 (Jun 01 2003) ISSN: 0006-4971 [Print] United States
• PMID: 12560240 (Publication Type: Case Reports, Journal Article)

Chemical References

• Phosphatidylserines

Topics

• Adult
• Anemia, Hemolytic, Congenital (etiology, pathology)
• Case-Control Studies
• Cell Adhesion
• Child
• Endothelium, Vascular (cytology)
• Erythrocyte Membrane (physiology, ultrastructure)
• Erythrocytes (pathology)
• Family Health
• Humans
• Male
• Phosphatidylserines
• Thrombosis (etiology)
• Umbilical Veins (cytology)