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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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-10-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1269325/full |
| _version_ | 1797668192441597952 |
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| author | Nicole Galli Nicole Galli Loredana Pettine Mauro Panigada Laura Daprai Laura Daprai Grazia Suriano Anna Grancini Anna Grancini Wilma Barcellini Bruno Fattizzo Bruno Fattizzo |
| author_facet | Nicole Galli Nicole Galli Loredana Pettine Mauro Panigada Laura Daprai Laura Daprai Grazia Suriano Anna Grancini Anna Grancini Wilma Barcellini Bruno Fattizzo Bruno Fattizzo |
| author_sort | Nicole Galli |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-11T20:24:59Z |
| format | Article |
| id | doaj.art-1ea51b8f8a6c49dcaca2b973e0841afb |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-03-11T20:24:59Z |
| publishDate | 2023-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-1ea51b8f8a6c49dcaca2b973e0841afb2023-10-02T15:34:52ZengFrontiers Media S.A.Frontiers in Immunology1664-32242023-10-011410.3389/fimmu.2023.12693251269325Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literatureNicole Galli0Nicole Galli1Loredana Pettine2Mauro Panigada3Laura Daprai4Laura Daprai5Grazia Suriano6Anna Grancini7Anna Grancini8Wilma Barcellini9Bruno Fattizzo10Bruno Fattizzo11Ematologia, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyEmatologia, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyAnestesia e Terapia Intensiva Adulti, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyLaboratorio di Microbiologia, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyLaboratorio Analisi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyAnestesia e Terapia Intensiva Adulti, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyLaboratorio di Microbiologia, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyLaboratorio Analisi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyEmatologia, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyEmatologia, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyParoxysmal 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.https://www.frontiersin.org/articles/10.3389/fimmu.2023.1269325/fullparoxysmal nocturnal hemoglobinuriaeculizumabravulizumabNeisseria meningitidesproximal complement inhibitors |
| spellingShingle | Nicole Galli Nicole Galli Loredana Pettine Mauro Panigada Laura Daprai Laura Daprai Grazia Suriano Anna Grancini Anna Grancini Wilma Barcellini Bruno Fattizzo Bruno Fattizzo Non-capsulated Neisseria meningitidis sepsis in a paroxysmal nocturnal hemoglobinuria patient treated with ravulizumab: case report and review of the literature Frontiers in Immunology paroxysmal nocturnal hemoglobinuria eculizumab ravulizumab Neisseria meningitides proximal complement inhibitors |
| 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 | paroxysmal nocturnal hemoglobinuria eculizumab ravulizumab Neisseria meningitides proximal complement inhibitors |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1269325/full |
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