It takes two to thrombosis: Hemolysis and complement.

Delvasto-Nuñez, Laura; Jongerius, Ilse; Zeerleder, Sacha (2021). It takes two to thrombosis: Hemolysis and complement. (In Press). Blood reviews, p. 100834. Elsevier 10.1016/j.blre.2021.100834

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Thromboembolic events represent the most common complication of hemolytic anemias characterized by complement-mediated hemolysis such as paroxysmal nocturnal hemoglobinuria and autoimmune hemolytic anemia. Similarly, atypical hemolytic uremic syndrome is characterized by hemolysis and thrombotic abnormalities. The main player in the development of thrombosis in hemolytic diseases is suggested to be the complement system. However, the release of extracellular hemoglobin and heme by hemolysis itself can also drive procoagulant responses. Both, complement activation and hemolysis promote the activation of neutrophils resulting in the formation of neutrophil extracellular traps and induce inflammation and vascular damage which all together might (synergistically) lead to hypercoagulability. In this review we aim to summarize the current knowledge on the role of complement activation and hemolysis in the onset of thrombosis in hemolytic diseases. This review will discuss the interplay between different biological systems and neutrophil activation contributing to the pathogenesis of thrombosis. Finally, we will combine this fundamental knowledge and address the pathophysiology of hemolysis in prototypical complement-driven diseases.

Item Type:

Journal Article (Review Article)


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


600 Technology > 610 Medicine & health








Pierrette Durand Lüthi

Date Deposited:

28 Jun 2021 15:14

Last Modified:

29 Jun 2021 01:33

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Coagulation Complement Hemolysis NETs Neutrophils Thrombosis




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