Sukhu K, Poovalingam V, Mahomed R, Giangrande P L F
Oxford Haemophilia Centre and Thrombosis Unit, Churchill Hospital, Oxford, UK.
Clin Lab Haematol. 2003 Aug;25(4):247-9. doi: 10.1046/j.1365-2257.2003.00523.x.
The amount and function of von Willebrand factor (VWF), measured against a panel of laboratory tests, is the normal basis for the diagnosis of von Willebrand's disease (VWD). The normal range for each test is usually obtained by assaying samples from a cross section of the local population or from a manufacturer's kit insert. While collecting normal controls for another study, population from Durban in South Africa, with its distinct ethnic mix of Africans, Indians and Caucasians were also studied. Previously, Indians from their subcontinent have not been looked at separately and compared with Africans and Caucasians. It is confirmed in a previous study (Miller, Dilley, Richardson, Craig, Evatt. (2001) American Journal of Hematology 67, 125) that African Americans had significantly higher VWF:Ag and FVIII levels when compared with Caucasians. In addition, it was found that there was a significant difference in VWF:Ag levels between Indians and Africans, and between Caucasians and Africans, whereas no significant difference between Indians and Caucasians. Africans, Indians and Caucasians with blood group O showed significantly lower VWF:Ag and FVIII than the other ABO blood groups. Normal ranges of VWF for different blood groups are well established and this information should be utilized while considering a diagnosis of VWD. It is proposed here that the influence of racial origin should also be considered in the clinical and laboratory evaluation of VWD.
通过一系列实验室检测来测定血管性血友病因子(VWF)的含量和功能,是诊断血管性血友病(VWD)的常规依据。每项检测的正常范围通常是通过检测当地人群的横断面样本或制造商试剂盒说明书中的样本获得的。在为另一项研究收集正常对照时,还对南非德班的人群进行了研究,该人群有非洲人、印度人和高加索人独特的种族混合。此前,来自印度次大陆的印度人尚未被单独研究并与非洲人和高加索人进行比较。一项先前的研究(Miller, Dilley, Richardson, Craig, Evatt. (2001) American Journal of Hematology 67, 125)证实,与高加索人相比,非裔美国人的VWF:Ag和FVIII水平显著更高。此外,还发现印度人和非洲人之间、高加索人和非洲人之间的VWF:Ag水平存在显著差异,而印度人和高加索人之间没有显著差异。O血型的非洲人、印度人和高加索人的VWF:Ag和FVIII水平显著低于其他ABO血型。不同血型的VWF正常范围已得到充分确立,在考虑诊断VWD时应利用这些信息。本文提出,在VWD的临床和实验室评估中也应考虑种族起源的影响。