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Updated: Sep 23, 2025

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Postsurgical Thrombotic Microangiopathy and Deregulated Complement.

Thijs T W van Herpt1, Sjoerd A M E G Timmermans2, Walther N K A van Mook1,3

  • 1Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands.

Journal of Clinical Medicine
|May 14, 2022
PubMed
Summary
This summary is machine-generated.

Postsurgical thrombotic microangiopathy (TMA) is a serious complication. This case highlights complement activation

Keywords:
aortic surgerycomplement activationcomplement inhibitiongenetic variancehemolytic uremic syndromemultidisciplinary approachthrombocytopeniathrombotic micro-angiopathy

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Area of Science:

  • Nephrology
  • Hematology
  • Immunology

Background:

  • Postsurgical thrombotic microangiopathy (TMA) is a rare but serious complication following surgery, often presenting with acute kidney injury and thrombocytopenia.
  • The underlying pathophysiological mechanisms of postsurgical TMA remain largely unknown, leading to delayed diagnosis and treatment.
  • Early recognition and management are crucial due to the significant morbidity and mortality associated with this condition.

Observation:

  • A 56-year-old male developed anuric acute kidney injury, Coombs-negative hemolysis, and thrombocytopenia after surgical aortic arch replacement.
  • Massive ex vivo complement activation was observed on the endothelium.
  • The patient possessed a rare complement gene variant in C2 and an at-risk MCPggaac haplotype.

Findings:

  • Complement activation plays a pivotal role in the pathophysiology of postsurgical TMA.
  • Genetic predisposition, including C2 variants and MCP haplotypes, may contribute to disease development.
  • Therapeutic complement inhibition led to an excellent clinical response.

Implications:

  • This case underscores the importance of considering complement-mediated pathways in the diagnosis of postsurgical TMA.
  • Genetic screening for complement gene variants may aid in identifying at-risk individuals.
  • A multidisciplinary team approach is essential for managing complex postsurgical complications like TMA.