Abstract
Evidence supporting the existence of intravascular coagulation in homozygous sickle cell (HbSS) disease has been reported. In this study, surface-mediated reactions of clotting were compared in 21 black children with HbSS disease and 12 age-matched controls. Both the coagulant and antigen titers of Hageman factor were decreased (mean coagulant titer 0.49 ± S.D. 0.29 u/ml; mean antigen titer 0.77 ± 0.27 u/ml) in asymptomatic HbSS patients compared to the control group (mean coagulant titer. 1.01 ± 0.39 u/ml; mean antigen titer 0.94 ± 0.25 u/ml). A disparate relationship between the Hageman factor coagulant and antigen titers were observed in HbSS patients. These findings were associated with a slight decrease in the plasma titers of prekallikrein and HMW kininogen (mean 0.78 ± 0.14 and 0.75 ± 0.15 u/ml respectively in HbSS patients compared to 1.05 ± 0.22 and 0.86 ± 0.09 u/ml respectively in the control group). A further reduction from the initially low titers of these contact factors was observed during vaso-occlusive crises (mean Hageman factor titer 0.32 ± 0.15 u/ml; mean prekallikrein titer 0.38 ± 0.18 u/ml; mean HMW kininogen titer 0.56 ± 0.17 u/ml). These data indicate that the initial participants of the intrinsic pathway of coagulation are consumed in patients with HbSS disease. Further, these processes appear to accelerate during vaso-occlusive crises.
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Gordon, E., Klein, B., Berman, B. et al. 910 EVIDENCE FOR REDUCTION OF CONTACT FACTORS IN SICKLE CELL DISEASE. Pediatr Res 19, 262 (1985). https://doi.org/10.1203/00006450-198504000-00940
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DOI: https://doi.org/10.1203/00006450-198504000-00940
