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Published on: June 22, 2012
Thrombotic microangiopathies: An illustrated review
Mouhamed Yazan Abou-Ismail1, Sargam Kapoor2, Divyaswathi Citla Sridhar3
1Division of Hematology and Hematologic Malignancies Department of Internal Medicine University of Utah Health Sciences Center Salt Lake City Utah USA.
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.
Insights
Thrombotic microangiopathies (TMAs) are serious blood disorders. This review details their diagnosis and management, including emerging therapies for complement-mediated TMAs.
Area of Science:
- Hematology
- Pathophysiology
- Rare Diseases
Background:
- Thrombotic microangiopathies (TMAs) encompass diverse disorders causing thrombosis.
- TMAs present with microangiopathic hemolytic anemia, thrombocytopenia, and organ ischemia.
- These conditions carry significant morbidity and mortality, with evolving management strategies.
Purpose of the Study:
- To review the incidence, pathophysiology, diagnosis, and management of key TMAs.
- To focus on thrombotic thrombocytopenic purpura (TTP), Shiga toxin-associated HUS, and complement-mediated HUS.
- To highlight novel complement-directed therapies for TMAs.
Main Methods:
- Illustrated review of TMAs.
- Focus on specific TMA subtypes: TTP, STX-HUS, c-HUS, HCT-TMA, drug-induced TMA.
- Discussion of diagnostic evaluations and therapeutic approaches.
Main Results:
- Detailed descriptions of the incidence, pathophysiology, diagnosis, and management of selected TMAs.
- Identification of emerging complement-directed therapies for complement-mediated TMAs.
- Emphasis on the importance of accurate diagnosis for effective management.
Conclusions:
- Accurate diagnosis is crucial for managing heterogeneous TMAs.
- Management strategies for TMAs are continually evolving.
- Emerging therapies show promise for specific TMA subsets, particularly complement-mediated forms.
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