Hematology· Topic 6.2 Anemia from hemolysis and abnormal hemoglobin· 6.2.4

Hemolytic uremic syndrome

Hemolytic uremic syndrome is a thrombotic microangiopathy defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, most often triggered in children by Shiga toxin-producing Escherichia coli O157:H7 after bloody diarrhea. Diagnosis rests on schistocytes with a negative Coombs test plus a normal ADAMTS13 level to exclude TTP, and typical STEC-HUS is managed supportively while antibiotics and antimotility agents are avoided, whereas complement-mediated atypical HUS requires eculizumab.

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What this article covers

  • Overview

    Definition: hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy in which platelet-rich microthrombi form in small vessels, producing the triad of MAHA, thrombocytopenia, and acute kidney injury (AKI).

  • Epidemiology

    Predominantly a pediatric disease: classic diarrhea-associated (D+) HUS chiefly affects children, following ingestion of undercooked meat or other food contaminated with E.

  • Risk factors

    Ingestion of undercooked meat or contaminated food or water: the principal exposure for Shiga toxin-producing E.

  • Pathophysiology

    Toxin-mediated endothelial injury (typical HUS): Shiga-like toxin from E.

  • Clinical features

    Preceding bloody diarrhea (typical HUS): a diarrheal prodrome from Shiga toxin-producing E.

  • Differential diagnosis

    Thrombotic thrombocytopenic purpura: more prominent fever and fluctuating neurologic signs than renal failure, and ADAMTS13 below 10% (thrombotic thrombocytopenic purpura).

  • Diagnosis

    Establish the TMA triad, then confirm the mechanism: the diagnosis rests on MAHA (schistocytes, elevated LDH, decreased haptoglobin, elevated indirect bilirubin and reticulocyte count, negative…

  • Management

    Start with supportive care for typical (STEC) HUS, the mainstay of treatment: IV fluids and electrolyte replacement to manage volume status and correct derangements from the preceding diarrhea, with…

  • Complications

    Acute kidney injury progressing to dialysis-dependent renal failure: the dominant acute complication, particularly in adults and severe pediatric cases.

  • Prognosis

    Typical (D+) HUS in children carries the best prognosis of the group, with most recovering kidney function (see Epidemiology for figures); adults with D+HUS fare worse.

  • Prevention

    Food safety to prevent STEC exposure: thoroughly cook ground meat (no pink color, killing E.

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