Moorehead Paul C, Thibeault Lisa, Tuttle Angie, Grabell Julie, Dwyre Louise, Silva Mariana, James Paula, Lillicrap David
*Janeway Children's Health and Rehabilitation Centre, St. John's, NF †Kingston General Hospital ‡Faculty of Health Sciences, Queen's University, Kingston, ON, Canada.
J Pediatr Hematol Oncol. 2015 May;37(4):e220-2. doi: 10.1097/MPH.0000000000000287.
We report an 11-month-old boy with severe hemophilia A who had regular exposure to factor VIII (FVIII) intended to reduce the risk of developing an inhibitor. He developed a high-titer inhibitor (peak titer 19 BU) that disappeared within 6 weeks of starting immune tolerance induction (ITI). Anti-FVIII IgG4 peaked briefly compared with anti-FVIII IgG1 and the Bethesda titer. Neither rapid resolution of an inhibitor after prophylaxis nor this behavior of anti-FVIII IgG4 has been previously reported. Transient anti-FVIII IgG4 may be a marker of an attenuated anti-FVIII response induced by prophylactic FVIII therapy.
我们报告了一名11个月大的重度甲型血友病男孩，他定期接受凝血因子VIII（FVIII）治疗，目的是降低产生抑制剂的风险。他产生了高滴度的抑制剂（峰值滴度为19 BU），该抑制剂在开始免疫耐受诱导（ITI）后的6周内消失。与抗FVIII IgG1和贝塞斯达滴度相比，抗FVIII IgG4短暂达到峰值。此前尚未报道过预防后抑制剂的快速消退以及抗FVIII IgG4的这种表现。短暂的抗FVIII IgG4可能是预防性FVIII治疗诱导的抗FVIII反应减弱的一个标志。
