Centre for Haematology, Imperial College London, London, UK.
Br J Haematol. 2012 Aug;158(4):442-52. doi: 10.1111/j.1365-2141.2012.09201.x. Epub 2012 Jul 9.
The identification of variant Creutzfeldt-Jakob disease (vCJD) in the UK in 1996 led to significant concerns about the possibility of secondary transmission, however the prevalence of subclinical vCJD and risks of vCJD transmission by plasma are not known. In the UK, public health precautions have been implemented in all recipients of coagulation factor concentrates manufactured from UK plasma pools between 1980 and 2001. The recent demonstration of abnormal prion protein in a spleen sample at autopsy of a UK haemophilic patient who received coagulation factor concentrates to which a donor incubating vCJD had contributed most likely represents the first case of vCJD transmission by coagulation factor concentrates. We review the uncertainties that surround risk of vCJD transmission by coagulation factor concentrates, the challenges in dealing with undefined risks, the rationale behind current policies and the implementation of vCJD surveillance and risk management measures in bleeding disorder patients in the UK.
1996 年在英国发现变异型克雅氏病（vCJD）后，人们非常担心该病可能会发生二次传播，然而，目前尚不清楚亚临床 vCJD 的流行程度以及通过血浆传播 vCJD 的风险。在英国，自 1980 年至 2001 年期间，所有使用源自英国血浆池的凝血因子浓缩物的接受者都采取了公共卫生预防措施。最近，在一名接受凝血因子浓缩物治疗的英国血友病患者的尸检脾脏样本中发现了异常朊病毒蛋白，这极有可能代表首例通过凝血因子浓缩物传播的 vCJD 病例。我们对通过凝血因子浓缩物传播 vCJD 的风险所存在的不确定性进行了综述，包括处理未明风险所面临的挑战、现行政策背后的原理以及在英国出血性疾病患者中实施 vCJD 监测和风险管理措施的情况。
