Complement Activation and Inhibition in Autoimmune Hemolytic Anemia: Focus on Cold Agglutinin Disease

Seminars in Hematology
Sigbjørn Berentsen

Abstract

The classical complement pathway and, to some extent, the terminal pathway, are involved in the immune pathogenesis of autoimmune hemolytic anemia (AIHA). In primary cold agglutinin disease (CAD), secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria, the hemolytic process is entirely complement dependent. Complement activation also plays an important pathogenetic role in some warm-antibody AIHAs, especially when immunoglobulin M is involved. This review describes the complement-mediated hemolysis in AIHA with a major focus on CAD, in which activation of the classical pathway is essential and particularly relevant for complement-directed therapy. Several complement inhibitors are candidate therapeutic agents in CAD and other AIHAs, and some of these drugs seem very promising. The relevant in vitro findings, early clinical data and future perspectives are reviewed.

Citations

Nov 19, 2019·Blood Advances·Sarah A Lau-BraunhutMatt S Zinter
Jun 18, 2020·Research and Practice in Thrombosis and Haemostasis·Catherine M BroomeAdam Rosenthal
Aug 15, 2019·The New England Journal of Medicine·Robert A Brodsky
Sep 17, 2019·Therapeutic Advances in Hematology·Sigbjørn BerentsenMarc Michel
Sep 10, 2020·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Blake R RushingBrandon L Garcia
Jan 19, 2021·International Journal of Laboratory Hematology·Zhao WangYuping Zhao
Nov 2, 2019·Transfusion Medicine Reviews·Lillemor Skattum
Mar 11, 2021·Pharmacological Reviews·Peter GarredTom E Mollnes
Apr 8, 2021·The New England Journal of Medicine·Alexander RöthSigbjørn Berentsen
May 1, 2021·Journal of Clinical Medicine·Marco CapecchiIda Martinelli
Jul 1, 2021·Seminars in Immunopathology·M JalinkI Jongerius
Jul 27, 2021·Current Medical Research and Opinion·Emese K VágóJun Su
Aug 3, 2021·Experimental and Therapeutic Medicine·Jingwei Liu, Yumei Li

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