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Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature

Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired haematopoietic stem cell disease characterized by complement-mediated intravascular hemolysis, thrombosis, and bone marrow failure. Eculizumab and ravulizumab are anti-C5 monoclonal antibodies that reduce hemolysis, anaemia and thrombotic...

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Autores principales: Galli, Nicole, Pettine, Loredana, Panigada, Mauro, Daprai, Laura, Suriano, Grazia, Grancini, Anna, Barcellini, Wilma, Fattizzo, Bruno
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10580757/
https://www.ncbi.nlm.nih.gov/pubmed/37854608
http://dx.doi.org/10.3389/fimmu.2023.1269325
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author Galli, Nicole
Pettine, Loredana
Panigada, Mauro
Daprai, Laura
Suriano, Grazia
Grancini, Anna
Barcellini, Wilma
Fattizzo, Bruno
author_facet Galli, Nicole
Pettine, Loredana
Panigada, Mauro
Daprai, Laura
Suriano, Grazia
Grancini, Anna
Barcellini, Wilma
Fattizzo, Bruno
author_sort Galli, Nicole
collection PubMed
description Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired haematopoietic stem cell disease characterized by complement-mediated intravascular hemolysis, thrombosis, and bone marrow failure. Eculizumab and ravulizumab are anti-C5 monoclonal antibodies that reduce hemolysis, anaemia and thrombotic risk, but are associated with increased risk of infection with encapsulated bacteria, including Neisseria meningitidis. We report a case of life-threatening infection by non-groupable Neisseria meningitidis in a young PNH patient treated with ravulizumab. Despite prompt admission to the intensive care unit, microbe isolation was delayed due to the negativity of capsular antigens, and the patient required intubation, dialysis, and transfusion support for pancytopenia. Notably, PNH disease activity remained controlled and no additional anti-C5 doses were administered. Increasing awareness regarding septic risk in PNH patients on complement inhibitors despite vaccinations is pivotal. A warning about serotypes generally not pathogenetic and not covered by vaccination, such as non-capsulated forms, is emerging.
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spelling pubmed-105807572023-10-18 Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature Galli, Nicole Pettine, Loredana Panigada, Mauro Daprai, Laura Suriano, Grazia Grancini, Anna Barcellini, Wilma Fattizzo, Bruno Front Immunol Immunology Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired haematopoietic stem cell disease characterized by complement-mediated intravascular hemolysis, thrombosis, and bone marrow failure. Eculizumab and ravulizumab are anti-C5 monoclonal antibodies that reduce hemolysis, anaemia and thrombotic risk, but are associated with increased risk of infection with encapsulated bacteria, including Neisseria meningitidis. We report a case of life-threatening infection by non-groupable Neisseria meningitidis in a young PNH patient treated with ravulizumab. Despite prompt admission to the intensive care unit, microbe isolation was delayed due to the negativity of capsular antigens, and the patient required intubation, dialysis, and transfusion support for pancytopenia. Notably, PNH disease activity remained controlled and no additional anti-C5 doses were administered. Increasing awareness regarding septic risk in PNH patients on complement inhibitors despite vaccinations is pivotal. A warning about serotypes generally not pathogenetic and not covered by vaccination, such as non-capsulated forms, is emerging. Frontiers Media S.A. 2023-10-02 /pmc/articles/PMC10580757/ /pubmed/37854608 http://dx.doi.org/10.3389/fimmu.2023.1269325 Text en Copyright © 2023 Galli, Pettine, Panigada, Daprai, Suriano, Grancini, Barcellini and Fattizzo https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Galli, Nicole
Pettine, Loredana
Panigada, Mauro
Daprai, Laura
Suriano, Grazia
Grancini, Anna
Barcellini, Wilma
Fattizzo, Bruno
Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
title Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
title_full Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
title_fullStr Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
title_full_unstemmed Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
title_short Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
title_sort non-capsulated neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10580757/
https://www.ncbi.nlm.nih.gov/pubmed/37854608
http://dx.doi.org/10.3389/fimmu.2023.1269325
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