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Atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA), is a rare, life-threatening, systemic disease.
aHUS can be distinguished from TTP on the basis of ADAMTS13 activity, with a severe decrease (<5%-10% of control) characteristic of TTP1. It is important to order this test in any patient presenting with laboratory and clinical signs of a TMA before plasma therapy is initiated.
STEC-HUS can be ruled out using both culture and PCR-based assays of stool samples or rectal swabs for Shiga toxin-producing E. coli.
aHUS is diagnosed by exclusion, diagnosis should be oriented toward aHUS if disseminated intravascular coagulation is ruled out, STEC test is negative, and plasma activity of ADAMTS13 is > 10%.
Consider a multidisciplinary approach to diagnose and manage aHUS.
Acronyms:
References
Laurence J et al. Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol. 2016 Nov;14 Suppl 11(11):2-15.
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Azoulay E et al. Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uremic syndrome. Chest. Vol 152, Issue 2:424-434.
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