Altered erythrocyte endothelial adherence and membrane phospholipid asymmetry in hereditary hydrocytosis
Open Access
- 1 June 2003
- journal article
- case report
- Published by American Society of Hematology in Blood
- Vol. 101 (11), 4625-4627
- https://doi.org/10.1182/blood-2001-12-0329
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 β-thalassemia and may play a similar role in hereditary hydrocytosis.Keywords
This publication has 16 references indexed in Scilit:
- Thrombosis in heritable hemolytic disordersCurrent Opinion in Hematology, 1999
- Role of red blood cells in thrombosisCurrent Opinion in Hematology, 1999
- Hereditary dehydrated and overhydrated stomatocytosis: recent advancesCurrent Opinion in Hematology, 1999
- Increased erythrocyte phosphatidylserine exposure in sickle cell disease: flow-cytometric measurement and clinical associationsBlood, 1996
- THROMBO‐EMBOLIC DISEASE AFTER SPLENECTOMY FOR HEREDITARY STOMATOCYTOSISBritish Journal of Haematology, 1996
- Osmotic Scan Ektacytometry in Clinical DiagnosisJournal of Pediatric Hematology/Oncology, 1996
- Further investigations of red cell deformability with nickel meshBiorheology, 1990
- [2] Ektacytometry of red blood cellsMethods in enzymology, 1989
- Osmotic gradient ektacytometry: comprehensive characterization of red cell volume and surface maintenanceBlood, 1983
- Congenital Hemolytic Anemia with High Sodium, Low Potassium Red CellsNew England Journal of Medicine, 1968