New England Journal of Medicine · 1980 · 519 citations · 19 references
MedicineHematologyPathologyHematology PhysiologyHematopathologyHemostasisAnemiaEndothelial DysfunctionHematologic DisordersRed Blood Cell DisordersRed Blood Cell PhysiologySickle Cell DiseaseClinical Severity ClassificationClinical Severity ScoringErythrocyte AggregationMicrovascular HemodynamicsRed Blood Cell Hemoglobin ConcentrationReticulocyte IndicesSickle Cell Anemia
We studied 33 patients with sickle-cell anemia to examine the possible relation between the severity of their disease (frequency of microvascular occlusions) and the abnormal adherence of sickle erythrocytes to cultured human endothelium. Neither clinical severity nor erythrocyte adherence correlates significantly with red-cell indexes, hemoglobin concentration, percentage of irreversibly sickled red cells, level of fetal hemoglobin, or reticulocyte count. However, clinical severity and erythrocyte adherence are strongly correlated (rank correlation coefficient = +0.666; P less than 0.001). These findings are consistent with the hypothesis that abnormal interactions between erythrocytes and endothelium may be the initiating factor in the development of microvascular occlusions in sickle-cell anemia.
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Abnormal Adherence of Sickle Erythrocytes to Cultured Vascular Endothelium
Robert P. Hebbel, Osamu Yamada, Charles F. Moldow et al. · Journal of Clinical Investigation · 1980 · 421 citations · Full text
Medicine, Cell Biology, Hematology +15
Studies on Abnormal Hemoglobins
Karl Singer, Ben Fisher · Blood · 1952 · 192 citations · Full text