Annals of Hematology

, Volume 96, Issue 4, pp 589–596 | Cite as

Serologic response to meningococcal vaccination in patients with paroxysmal nocturnal hemoglobinuria (PNH) chronically treated with the terminal complement inhibitor eculizumab

  • Ferras Alashkar
  • Colin Vance
  • Dörte Herich-Terhürne
  • Nicole Preising
  • Ulrich Dührsen
  • Alexander Röth
Original Article


Eculizumab is indicated for the therapy of patients with symptomatic paroxysmal nocturnal hemoglobinuria (PNH). Due to inhibition of terminal complement cascade, patients on eculizumab are susceptible to Neisseria meningitidis infections. The two mainstays to reduce the risk of infection are vaccination and antibiotic prophylaxis. In this retrospective study, serologic response was analyzed after vaccination with a meningococcal vaccine in 23 PNH patients (median age 36 years; range 25 - 88 years; 15 males, 8 females) by measuring serum bactericidal assay (SBA) using rabbit complement (rSBA) titers against meningococcal serogroups A, C, W, and Y. Serologic protection was defined by an rSBA titer ≥1:8. Forty-three percent (10/23) were vaccinated more than once due to chronic eculizumab treatment. Overall serologic response for 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 response to vaccines varies, the use of serologic response analyses is warranted. Re-vaccination with a tetravalent conjugate vaccine under eculizumab therapy every 3 years is essential or should be based on response rates. If meningococcal infection is suspected, standby therapy with ciprofloxacin and immediate medical evaluation are recommended. The novel vaccines covering serogroup B may even further reduce the risk for infection.


PNH Meningococcal vaccination Eculizumab 



Paroxysmal nocturnal hemoglobinuria


Serum bactericidal assay (SBA) using rabbit complement (rSBA)


Compliance with ethical standards

Conflict of interest

The authors have indicated that they have no conflicts of interest regarding the content of this article.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Department of Hematology, West German Cancer CenterUniversity Hospital Essen, University of Duisburg-EssenEssenGermany
  2. 2.Rheinisch-Westfälisches Institut für WirtschaftsforschungEssenGermany

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