POSTERS

摘要

The hallmark of sickle cell disease (SCD) is the presence of distorted, rigid red blood cells (RBC) in the circulation that, under certain conditions, can promote painful microvascular occlusion. Although the exact pathomechanisms are not yet known, there is evidence suggesting that in addition to reduced RBC deformability, other hemorheological alterations may contribute to the clinical manifestations of vasoocclusive crisis. The present study was designed to compare RBC deformability and aggregation indices between healthy controls (AA genotype, n = 10) and patients with either homozygous (SS genotype, n = 17) or heterozygous SCD (SC genotype, n = 13). All patients were in steady state and had not been transfused for at least 90 days prior to enrollment. RBC deformability as an elongation index (El) was measured at 3 and 30 Pa using the LORCA laser diffraction system. The LORCA was also utilized to obtain the following aggregation parameters: (1) aggregation index (AI); (2) aggregation half time (t~(1/2)); (3) the minimal shear stress required to inhibit aggregate formation (7). In addition, routine hematological parameters and plasma fibrinogen levels were determined.