Pediatric Infectious Diseases and Immunology, Amalia Children's Hospital, Nijmegen, the Netherlands.
Expertise Center for Immunodeficiency and Autoinflammation (REIA), Radboudumc, Nijmegen, the Netherlands.
Clin Exp Immunol. 2019 Dec;198(3):381-389. doi: 10.1111/cei.13368. Epub 2019 Oct 1.
Vaccination against meningococcal serogroup B is recommended for patients with a complement deficiency; however, although immunogenicity in this patient group has been shown, efficacy has not yet been established. In this study, we collected serum from children with a complement deficiency in the alternative pathway or in late terminal pathway before and after vaccination with multi-component meningococcal serogroup B (MenB)-4C. MenB-4C is a multi-component, protein-based vaccine against MenB consisting of factor H-binding protein, Neisserial heparin-binding protein, Neisserial adhesion A and outer membrane vesicles containing Porin A. We assessed the vaccine immunogenicity and vaccine-mediated protection by a whole cell enzyme-linked immunosorbent assay with Neisseria meningitidis serogroup B strains H44/76, 5/99 and NZ98/254, which shows that vaccination induced antibody titers against meningococcus. We show that the classical serum bactericidal activity assay with exogenous serum indicates the presence of vaccine-induced antibodies and capacity to activate complement-mediated pathogen lysis. However, in children with a late terminal pathway deficiency, no complement-mediated pathogen lysis was observed when autologous serum was applied in the serum bactericidal activity assay, demonstrating a lack of serum bactericidal activity in children with complement deficiencies. However, MenB-4C vaccination still induced effective complement-dependent opsonophagocytic killing against N. meningitidis serogroup B in reconstituted whole blood with autologous serum from children with an alternative pathway or late terminal pathway deficiency. These findings support the recommendation to vaccinate all complement-deficient children against MenB.
针对补体缺陷患者,推荐接种脑膜炎奈瑟菌 B 型多价疫苗;然而,虽然该患者群体的免疫原性已得到证实,但尚未确定其疗效。在这项研究中,我们收集了替代途径或晚期终端途径补体缺陷儿童在接种多组分脑膜炎奈瑟菌 B 型(MenB)-4C 前后的血清。MenB-4C 是一种多组分、基于蛋白质的针对 MenB 的疫苗,包含因子 H 结合蛋白、奈瑟菌肝素结合蛋白、奈瑟菌黏附 A 和含有 Porin A 的外膜囊泡。我们通过使用脑膜炎奈瑟菌 B 血清型 H44/76、5/99 和 NZ98/254 菌株的全细胞酶联免疫吸附试验评估了疫苗的免疫原性和疫苗介导的保护作用,该试验表明接种疫苗可诱导针对脑膜炎球菌的抗体滴度。我们表明,外源性血清的经典血清杀菌活性测定表明存在疫苗诱导的抗体和激活补体介导的病原体裂解的能力。然而,在晚期终端途径缺陷的儿童中,当在血清杀菌活性测定中应用自身血清时,未观察到补体介导的病原体裂解,表明补体缺陷儿童的血清杀菌活性缺乏。然而,MenB-4C 疫苗接种仍然在含有替代途径或晚期终端途径缺陷儿童自身血清的重建全血中诱导针对脑膜炎奈瑟菌 B 血清型的有效补体依赖性调理吞噬杀伤作用。这些发现支持对所有补体缺陷儿童接种 MenB 的建议。