Serologic response to meningococcal vaccination in patients with paroxysmal nocturnal hemoglobinuria (PNH) chronically treated with the terminal complement inhibitor eculizumab
Eculizumab is indicated for the therapy of patients with symptomatic paroxysmalnocturnal hemoglobinuria(PNH). Due to inhibition of terminal complement cascade, patients on eculizumab are susceptible to Neisseria meningitidisinfections. The two mainstays to reduce the risk of infection arevaccination and antibiotic prophylaxis. In this retrospectivestudy, serologic response was analyzed after vaccination witha meningococcal vaccine in 23 PNH patients (median age36 years; range 25 - 88 years; 15 males, 8 females) by measuringserum bactericidal assay (SBA) using rabbit complement(rSBA) titers against meningococcal serogroups A, C, W, andY. Serologic protection was defined by an rSBA titer≥1:8.Fortythree percent (10/23) were vaccinated more than oncedue to chronic eculizumab treatment. Overall serologic responsefor the meningococcal serogroups was A: 78% (18/23), C: 87%(20/23), W: 48% (11/23), and Y: 70% (16/23). No meningococcal infections have been observed. As immunological responseto vaccines varies, the use of serologic response analyses iswarranted. Re-vaccination witha tetravalent conjugate vaccineunder eculizumab therapy every 3 years is essential or should bebased on response rates. If meningococcal infection issuspected, standby therapy with ciprofloxacin andimmediate medical evaluation are recommended. The novelvaccines covering serogroup B may even further reduce the riskfor infection.
Alashkar, F., C. Vance Ph.D., D. Herich‐Terhürne, N. Preising, U. Dührsen und A. Röth (2017), Serologic response to meningococcal vaccination in patients with paroxysmal nocturnal hemoglobinuria (PNH) chronically treated with the terminal complement inhibitor eculizumab. Annals of Hematology, 96,4, 589-596