Department of Pediatric Hematology, Oncology and Hemostasis, Johann Wolfgang Goethe University Hospital, Frankfurt, Germany.
Transfusion. 2010 Jul;50(7):1571-80. doi: 10.1111/j.1537-2995.2010.02604.x. Epub 2010 Feb 26.
The modulation of Factor (F)VIII activity (FVIII : C), von Willebrand factor antigen (VWF : Ag), and von Willebrand factor ristocetin cofactor (VWF : RCo) by the ABO(H) blood group is well established in adults. Expression of ABH antigens on N-linked glycans of VWF protects plasma VWF from proteolysis and clearance. Protection by H antigens is less effective than by AB antigens, resulting in approximately 25% lower VWF plasma levels in adults with blood group O compared to non-O. Given the reduced branching of ABO(H) bearing structures (I blood group system) with lower numbers of H, A, and B antigen sites during the first 18 months of life, we reasoned that if the relationship between ABO(H) blood group and VWF levels were causal, the difference of ABO(H) blood group-dependent VWF levels should be marginal or not be observed in the first months of life.
We undertook quantification of FVIII : C and VWF in 574 presumably healthy children aged 1 to 210 months and correlated the values with ABO(H) blood type. Moreover, we establish reference intervals for common coagulation variables for several pediatric age groups.
Significant differences between blood group O versus non-O values of FVIII : C, VWF : Ag, and VWF : RCo were not observed in the first months of life, started to develop during childhood, and in adolescence reached adult values.
In comparison to the levels for adults and adolescents, we report fundamental differences of VWF levels in the first year of life, which may be associated with the physiologic development of the ABO(H) and I blood group system.
ABO(H) 血型对因子 (F)VIII 活性 (FVIII:C)、血管性血友病因子抗原 (VWF:Ag) 和血管性血友病因子瑞斯托菌素辅因子 (VWF:RCo) 的调节在成人中已得到充分证实。VWF 上 N 连接糖链上的 ABH 抗原可保护血浆 VWF 免受蛋白水解和清除。H 抗原的保护作用不如 AB 抗原有效,导致 O 型血成人的 VWF 血浆水平比非 O 型血低约 25%。鉴于在生命的前 18 个月中,ABO(H) 携带结构(I 血型系统)的分支减少,并且 H、A 和 B 抗原位点数量减少,因此我们推断,如果 ABO(H) 血型与 VWF 水平之间的关系是因果关系,那么在生命的前几个月中,ABO(H) 血型依赖性 VWF 水平的差异应该是微不足道的或不会被观察到。
我们对 574 名年龄在 1 至 210 个月的健康儿童进行了 FVIII:C 和 VWF 的定量检测,并将这些值与 ABO(H) 血型相关联。此外,我们为几个儿科年龄组建立了常见凝血变量的参考区间。
在生命的前几个月中,O 型血与非 O 型血的 FVIII:C、VWF:Ag 和 VWF:RCo 值之间没有显著差异,这种差异在儿童期开始出现,并在青春期达到成人值。
与成人和青少年的水平相比,我们报告了生命第一年 VWF 水平的根本差异,这可能与 ABO(H) 和 I 血型系统的生理发育有关。