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A previously treated severe haemophilia A patient developed high-titre inhibitor after vaccinations.
International Journal of Immunopathology and Pharmacology ( IF 3.5 ) Pub Date : 2020-07-06 , DOI: 10.1177/2058738420934618
Zekun Li 1 , Zhenping Chen 1 , Xiaoling Cheng 1 , Xinyi Wu 1 , Gang Li 1 , Yingzi Zhen 1 , Man-Chiu Poon 2 , Runhui Wu 1
Affiliation  

The factor VIII (FVIII)-neutralizing antibody (inhibitor) seen in 25%–30% of patients with severe haemophilia A (SHA). Vaccination is a non-genetic risk factor of inhibitor development as ‘danger signal’ which may provide a pro-inflammatory microenvironment to increase FVIII immunogenicity. We reported a previously treated SHA patient postponed the first vaccination to 15-month age received diphtheria-pertussis-tetanus intramuscularly. At 18-month age, the patient received Hepatitis A intramuscularly and Varicella Zoster Virus subcutaneously with 2 weeks interval and FVIII infusion was given <24 h prior for each. Successive bleedings occurred 1 week later with inefficacy of FVIII replacement. High-titre inhibitor was tested at 117 exposure days. This case suggested that continuous vaccinations in close proximity to FVIII could induce inhibitor. The relationship between vaccination and FVIII immunogenicity still needs to be revealed by further study.



中文翻译:

先前治疗过的严重血友病A患者在接种疫苗后出现了高滴度抑制剂。

严重A型血友病(SHA)患者中有25%–30%观察到VIII因子(FVIII)中和抗体(抑制剂)。疫苗接种是抑制剂发展的非遗传危险因素,称为“危险信号”,可提供促炎性微环境以增加FVIII免疫原性。我们报道了以前接受过治疗的SHA患者肌肉注射白喉-百日咳-破伤风疫苗后将首次疫苗接种推迟到15个月大。在18个月大时,患者每隔2周进行一次皮下注射A型肝炎和水痘带状疱疹病毒,并且每一次均在<24小时内进行FVIII输注。1周后连续出血,FVIII替代无效。在117天暴露时测试了高滴度抑制剂。该病例表明在FVIII附近连续接种疫苗可能会诱导抑制剂。

更新日期:2020-07-06
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