What are TMAs?
Thrombotic microangiopathies (TMAs) are a group of severe and potentially life-threatening rare disorders that cause blood clots and damage to the walls of the smallest blood vessels (capillaries and small arteries) in the circulatory system. The blood clots can cause injury to organs that may lead to organ failure and death.1
In some cases, overactivation or dysregulation of the complement system can drive or worsen development of TMA. This overactivation fuels an attack on organs and cells in the body, including endothelial cells that line blood vessels.1
What is aHUS?
aHUS is a type of TMA that is caused by dyregulation of the complement system, which can occur due to genetic or environmental factors.6
aHUS may appear in the presence or absence of a trigger, or coexisting condition. aHUS is a rare disease that often presents suddenly with potentially severe complications and frequently develops into a progressive, chronic condition with relapses. 6-9
Signs, symptoms and complications of TMA include:
References.3-5
Anaemia
Thrombosis (blood clots)
Organ damage
including kidneys, brain and heart
Confusion
Shortness of breath
High blood pressure
Fatigue
References
- Brocklebank V, et al. Thrombotic microangiopathy and the kidney. Clin J Am Soc Nephrol. 2018;13:300-317.
- Cedzyński M, et al. Editorial: the role of complement in health and disease. Front Immunol. 2019;10:1869.
- Raina R, et al. Atypical hemolytic-uremic syndrome: an update on pathophysiology, diagnosis, and treatment. Ther Apher Dial. 2019;23(1):4-21.
- Sallée M, et al. Myocardial infarction is a complication of factor H-associated atypical HUS. Nephrol Dial Transplant. 2010;25(6):2028-2032.
- Laurence J, et al. Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol. 2016;11(11):2-15.
- Fakhouri F, et al. How I diagnose and treat atypical hemolytic uremic syndrome. Blood. 2023;141(9):984-995.
- Fremeaux-Bacchi V, et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 2013;8(4):554-562.
- Bayer G, et al. Etiology and outcomes of thrombotic microangiopathies. Clin J Am Soc Nephrol. 2019;14(4):557-566.
- Henry N, et al. Retrospective And Systematic Analysis Of Causes And Outcomes Of Thrombotic Microangiopathies In Routine Clinical Practice: an 11-year study. Front Med. 2021;8:566678.
- Cunningham JM, et al. Presentation and time to treatment of atypical hemolytic uremic syndrome is influenced by complement amplifying conditions: A single center experience. Blood. 2016;128(22):2543.
- George JN, et al. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654-66.
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