Reipert Birgit M, Hofbauer Christoph J, Gangadharan Bagirath, Berg Verena, Donnachie Elizabeth, Meeks Shannon, Mancuso Maria Elisa, Bowen Joel, Brown Deborah L
Baxalta Innovations GmbH, Takeda Company, 1220 Vienna, Austria.
Krems Bioanalytics, IMC University of Applied Sciences Krems, 3500 Krems an der Donau, Austria.
J Clin Med. 2023 Mar 6;12(5):2080. doi: 10.3390/jcm12052080.
Hemophilia A is a rare congenital bleeding disorder caused by a deficiency of functionally active coagulation factor VIII (FVIII). Most patients with the severe form of the disease require FVIII replacement therapies, which are often associated with the development of neutralizing antibodies against FVIII. Why some patients develop neutralizing antibodies while others do not is not fully understood. Previously, we could demonstrate that the analysis of FVIII-induced gene expression signatures in peripheral blood mononuclear cells (PBMC) obtained from patients exposed to FVIII replacement therapies provides novel insights into underlying immune mechanisms regulating the development of different populations of FVIII-specific antibodies. The aim of the study described in this manuscript was the development of training and qualification test procedures to enable local operators in different European and US clinical Hemophilia Treatment Centers (HTC) to produce reliable and valid data for antigen-induced gene expression signatures in PBMC obtained from small blood volumes. For this purpose, we used the model antigen Cytomegalovirus (CMV) phosphoprotein (pp) 65. We trained and qualified 39 local HTC operators from 15 clinical sites in Europe and the US, of whom 31 operators passed the qualification at first attempt, and eight operators passed at the second attempt.
甲型血友病是一种罕见的先天性出血性疾病,由功能性活性凝血因子VIII(FVIII)缺乏引起。大多数重型该病患者需要FVIII替代疗法,而这常常与产生抗FVIII的中和抗体有关。为何一些患者会产生中和抗体而另一些患者不会,目前尚未完全明确。此前,我们能够证明,对接受FVIII替代疗法患者外周血单个核细胞(PBMC)中FVIII诱导的基因表达特征进行分析,可为调控不同FVIII特异性抗体群体产生的潜在免疫机制提供新见解。本手稿所述研究的目的是开发培训和资格测试程序,使欧洲和美国不同临床血友病治疗中心(HTC)的当地操作人员能够针对从小血量获取的PBMC中的抗原诱导基因表达特征生成可靠且有效的数据。为此,我们使用了模型抗原巨细胞病毒(CMV)磷蛋白(pp)65。我们对来自欧洲和美国15个临床地点的39名当地HTC操作人员进行了培训和资格鉴定,其中31名操作人员首次尝试即通过资格鉴定,8名操作人员第二次尝试通过。