1st Medical Clinic and Polyclinic, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Department of Intensive Care Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Vaccine. 2018 May 3;36(19):2507-2509. doi: 10.1016/j.vaccine.2018.03.087. Epub 2018 Apr 7.
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired haematopoietic stem cell disease which causes defects in complement inhibiting proteins. The disease presents classically with the triad of haemolytic anaemia, pancytopenia and thrombosis. Eculizumab, a humanized antibody that blocks the cleavage of complement factor 5, was approved for PNH treatment in 2007 and has improved patients' survival since then. However, several cases of invasive meningococcal disease (IMD) have been reported in eculizumab-treated patients, mostly caused by serogroup B infection which was not covered by the previously administered vaccine (MenACWY). We report a rare case of septic shock due to infection with Neisseria meningitis serogroup B despite prior vaccination with 4CMenB in a young PNH patient treated with eculizumab. There are increasing doubts over whether vaccination ensures sufficient immunoprotection against IMD in patients receiving eculizumab. Therefore, besides monitoring the immune response, lifelong chemoprophylaxis should be considered.
阵发性睡眠性血红蛋白尿症（PNH）是一种罕见的获得性造血干细胞疾病，导致补体抑制蛋白缺陷。该疾病经典表现为溶血性贫血、全血细胞减少和血栓形成三联征。依库珠单抗是一种人源化抗体，可阻断补体因子 5 的裂解，于 2007 年被批准用于 PNH 治疗，自此改善了患者的生存率。然而，在接受依库珠单抗治疗的患者中已报告了几例侵袭性脑膜炎球菌病（IMD），主要由以前未接种的疫苗（MenACWY）覆盖的 B 群感染引起。我们报告了一例年轻 PNH 患者在接受依库珠单抗治疗的情况下，尽管先前接种了 4CMenB，但由于感染 B 群脑膜炎奈瑟菌而导致感染性休克的罕见病例。越来越多的人怀疑在接受依库珠单抗治疗的患者中，疫苗接种是否能确保对 IMD 有足够的免疫保护。因此，除了监测免疫反应外，还应考虑终身化学预防。
