Jalink, M; de Boer, E C W; Evers, D; Havinga, M Q; Vos, J M I; Zeerleder, S; de Haas, M; Jongerius, I (2021). Halting targeted and collateral damage to red blood cells by the complement system. Seminars in immunopathology, 43(6), pp. 799-816. Springer-Verlag 10.1007/s00281-021-00859-8
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The complement system is an important defense mechanism against pathogens; however, in certain pathologies, the system also attacks human cells, such as red blood cells (RBCs). In paroxysmal nocturnal hemoglobinuria (PNH), RBCs lack certain complement regulators which sensitize them to complement-mediated lysis, while in autoimmune hemolytic anemia (AIHA), antibodies against RBCs may initiate complement-mediated hemolysis. In recent years, complement inhibition has improved treatment prospects for these patients, with eculizumab now the standard of care for PNH patients. Current complement inhibitors are however not sufficient for all patients, and they come with high costs, patient burden, and increased infection risk. This review gives an overview of the underlying pathophysiology of complement-mediated hemolysis in PNH and AIHA, the role of therapeutic complement inhibition nowadays, and the high number of complement inhibitors currently under investigation, as for almost every complement protein, an inhibitor is being developed. The focus lies with novel therapeutics that inhibit complement activity specifically in the pathway that causes pathology or those that reduce costs or patient burden through novel administration routes.
Item Type: |
Journal Article (Review Article) |
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Division/Institute: |
04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory 04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Unit Childrens Hospital > Forschungsgruppe Hämatologie (Erwachsene) |
UniBE Contributor: |
Zeerleder, Sacha Sergio |
Subjects: |
600 Technology > 610 Medicine & health |
ISSN: |
1863-2297 |
Publisher: |
Springer-Verlag |
Language: |
English |
Submitter: |
Pierrette Durand Lüthi |
Date Deposited: |
26 Nov 2021 17:15 |
Last Modified: |
05 Dec 2022 15:55 |
Publisher DOI: |
10.1007/s00281-021-00859-8 |
PubMed ID: |
34191092 |
Uncontrolled Keywords: |
Autoimmune hemolytic anemia Complement Complement inhibitors Complement therapeutics Paroxysmal nocturnal hemoglobinuria |
BORIS DOI: |
10.48350/161609 |
URI: |
https://boris.unibe.ch/id/eprint/161609 |
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