Children with erythrocytosis due to cyanotic congenital heart diseases (CCHD) show an increased tendency to thrombotic disorders. We studied red cell deformability and 2,3-DPG concentration, platelet aggregation and platelet release in 18 children with CCHD ( PaO2 55%) was present in 3 patients. Red cell deformability was measured by a filtration technique. Platelet aggregation was assessed by the change in light transmission of platelet-rich plasma after addition of ADP (1-3 μM). Plasma β-TG level was determined by a radioimmunoassay, and 2,3-DPG by enzymatic analysis. Filtration time was 80±10 sec/ml (mean±SD) vs 30±8 sec/ml (p<0.01). 2,3-DPG was not significantly increased: 4.83±0.15 mmol/l erythrocytes (mean±SD) vs 4.18±0.1. In all samples platelet aggregation was impaired, while plasma β-TG was increased: 55±5μg/ml (mean±SD) vs 20±2, p<0.001. No correlation was demonstrated between red cell deformability and erythrocytosis or 2,3-DPG content. Our data show that rigid red cells and “exhausted” platelets (as suggested by impaired in vitro aggregation and increased plasma β-TG level) may occur in patients with CCHD also in absence of severe hypoxia.
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