SHM Converge Scientific Abstract ePoster Gallery-Breaking the Mimicry: Distinguishing Atypical Hus From TTP in an Acute Maha Presentation

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This document describes the challenge of distinguishing thrombotic thrombocytopenic purpura (TTP) from atypical hemolytic uremic syndrome (aHUS) in patients presenting with acute microangiopathic hemolytic anemia (MAHA). The case highlights that both conditions can look very similar at first, including findings such as schistocytes on peripheral smear, thrombocytopenia, renal injury, and purpuric skin lesions.<br /><br />The key clinical message is that early differentiation is essential because treatment differs substantially. TTP requires urgent plasma exchange, while aHUS is treated with complement inhibition, such as eculizumab. Delay in recognizing the correct diagnosis can worsen outcomes.<br /><br />Several practical diagnostic clues are emphasized. ADAMTS13 activity is the most important test for distinguishing the two: severe deficiency, especially with an inhibitor, confirms acquired TTP, whereas ADAMTS13 activity above 10% supports aHUS. TTP usually causes more severe thrombocytopenia with relatively milder renal dysfunction, while aHUS more often presents with marked renal failure. Low C3 levels and elevated terminal complement markers such as C5a and C5b-9 also favor aHUS.<br /><br />The conclusion stresses that hospitalists, who often see these critically ill patients first, should maintain a high index of suspicion, begin supportive care promptly, and order targeted testing early. Dialysis and plasma exchange may be needed initially while the diagnosis is clarified. Overall, the report underscores the importance of recognizing the clinical overlap between TTP and aHUS so that organ-saving treatment can be started without delay.

Asset Subtitle

Ramanpreet Johal

Meta Tag

Author List Adam Gerry, Amber Childers, Daneshka Rivera Miranda, Joey Bettencourt Jr., Madhu Murthy, Nickolle Alexandra Cruz Figueroa, Paramveer Bhugra, Ramanpreet Johal, Shreyesi Srivastava, Shrilkumar Bhatt

Category Clinical Vignettes

Concept Atypical Hemolytic Uremic Syndrome

Concept Thrombotic Thrombocytopenic Purpura

Concept Microangiopathic Hemolytic Anemia

Concept ADAMTS13

Concept ADAMTS13 Activity

Distinguished Non-Finalist

Presenter Organization Abrazo Health

Presenting Author Ramanpreet Johal

Track Adult

Keywords

thrombotic thrombocytopenic purpura

atypical hemolytic uremic syndrome

acute microangiopathic hemolytic anemia

ADAMTS13 activity

plasma exchange

eculizumab

complement inhibition

thrombocytopenia

renal injury

schistocytes

Atypical Hemolytic Uremic Syndrome

Thrombotic Thrombocytopenic Purpura

Microangiopathic Hemolytic Anemia

ADAMTS13

ADAMTS13 Activity