TY - JOUR
T1 - Postsurgical Thrombotic Microangiopathy and Deregulated Complement
AU - van Herpt, Thijs T W
AU - Timmermans, Sjoerd A M E G
AU - van Mook, Walther N K A
AU - van Bussel, Bas C T
AU - van der Horst, Iwan C C
AU - Maessen, Jos G
AU - Natour, Ehsan
AU - van Paassen, Pieter
AU - Heuts, Samuel
N1 - Publisher Copyright:
© 2022 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2022/4/29
Y1 - 2022/4/29
N2 - Postsurgical thrombotic microangiopathy (TMA) is a complication associated with significant morbidity and mortality. Still, the pathophysiological underlying mechanism of postsurgical TMA, a diagnosis often overlooked in postoperative patients with acute kidney injury and thrombocytopenia, is largely unknown. Here, we report the case of a 56-year-old male that developed anuric acute kidney injury, Coombs-negative hemolysis, and thrombocytopenia after surgical aortic arch replacement. Massive ex vivo complement activation on the endothelium, a rare complement gene variant in C2, at-risk haplotype MCPggaac, and excellent response to therapeutic complement inhibition, points to the pivotal role of complement in the pathophysiology of disease. Moreover, the importance of a multidisciplinary team approach in (postsurgical) thrombocytopenia is emphasized.
AB - Postsurgical thrombotic microangiopathy (TMA) is a complication associated with significant morbidity and mortality. Still, the pathophysiological underlying mechanism of postsurgical TMA, a diagnosis often overlooked in postoperative patients with acute kidney injury and thrombocytopenia, is largely unknown. Here, we report the case of a 56-year-old male that developed anuric acute kidney injury, Coombs-negative hemolysis, and thrombocytopenia after surgical aortic arch replacement. Massive ex vivo complement activation on the endothelium, a rare complement gene variant in C2, at-risk haplotype MCPggaac, and excellent response to therapeutic complement inhibition, points to the pivotal role of complement in the pathophysiology of disease. Moreover, the importance of a multidisciplinary team approach in (postsurgical) thrombocytopenia is emphasized.
KW - ACTIVATION
KW - ECULIZUMAB
KW - HEME
KW - HEMOLYTIC-UREMIC SYNDROME
KW - aortic surgery
KW - complement activation
KW - complement inhibition
KW - genetic variance
KW - hemolytic uremic syndrome
KW - multidisciplinary approach
KW - thrombocytopenia
KW - thrombotic micro-angiopathy
U2 - 10.3390/jcm11092501
DO - 10.3390/jcm11092501
M3 - Article
C2 - 35566627
SN - 2077-0383
VL - 11
JO - Journal of Clinical Medicine
JF - Journal of Clinical Medicine
IS - 9
M1 - 2501
ER -