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. 2022 Apr 22;6(3):e12708. doi: 10.1002/rth2.12708

Thrombotic microangiopathies: An illustrated review

Mouhamed Yazan Abou‐Ismail 1,✉, Sargam Kapoor 2, Divyaswathi Citla Sridhar 3, Lalitha Nayak 4, Sanjay Ahuja 5
PMCID: PMC9027164  PMID: 35615754

Abstract

The thrombotic microangiopathies (TMAs) are a heterogenous group of disorders with distinct pathophysiologies that cause occlusive microvascular or macrovascular thrombosis, and are characterized by microangiopathic hemolytic anemia, thrombocytopenia, and/or end‐organ ischemia. TMAs are associated with significant morbidity and mortality, and data on the management of certain TMAs are often lacking. The nomenclature, classification, and management of various TMAs is constantly evolving as we learn more about these rare syndromes. Thorough clinical and laboratory evaluation is essential to distinguish various TMAs and arrive at an accurate diagnosis, which is key for appropriate management. In this illustrated review, we focus on thrombotic thrombocytopenic purpura (TTP), Shiga toxin–associated hemolytic uremic syndrome, complement‐mediated hemolytic uremic syndrome, hematopoietic cell transplant‐associated TMA, and drug‐induced TMA, and describe their incidence, pathophysiology, diagnosis, and management. We also highlight emerging complement‐directed therapies under investigation for the management of complement‐mediated TMAs.

Keywords: atypical hemolytic uremic syndrome, hemolytic uremic syndrome, management, pathophysiology, thrombotic microangiopathies, thrombotic thrombocytopenic purpura

Essentials.

  • Thrombotic microangiopathies (TMAs) are a diverse group of rare, life‐threatening disorders.

  • We review the incidence, pathophysiology, evaluation, and management of various TMAs.

  • Thorough evaluation is essential to distinguish TMAs, as management relies on accurate diagnosis.

  • Several complement‐directed therapies are emerging in the treatment of complement‐mediated TMAs.

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RELATIONSHIP DISCLOSURE

The authors report no conflicts of interest.

AUTHOR CONTRIBUTIONS

MYA created all illustrations. MYA, SK, DCS, LN, and SA contributed to the scientific content and revised the manuscript.

ACKNOWLEDGMENTS

Created with BioRender.com. Micrographs of peripheral smear and kidney biopsy were taken and provided by Kristi Smock, MD, Division of Pathology at the University of Utah, ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, Utah.

Abou‐Ismail MY, Kapoor S, Citla Sridhar D, Nayak L, Ahuja S. Thrombotic microangiopathies: An illustrated review. Res Pract Thromb Haemost. 2022;6:e12708. doi: 10.1002/rth2.12708

Handling Editor: Dr Michelle Sholzberg

Funding information

This work did not receive any funding.

Contributor Information

Mouhamed Yazan Abou‐Ismail, Email: yazan.abou-ismail@hsc.utah.edu, @DoctorYazanA.

Divyaswathi Citla Sridhar, @DivyaCitlaMD.

Lalitha Nayak, @nayaklalitha1.

Sanjay Ahuja, @ahujadoc.

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