Diarrhea-associated hemolytic uremic syndrome with severe neurological manifestations treated with IgG depletion through immunoadsorption.

ABSTRACT

BACKGROUND: Diarrhea-associated hemolytic uremic syndrome (HUS) is characterized by acute kidney injury with microangiopathic hemolytic anemia and thrombocytopenia with a diarrhea prodrome, typically caused by Shiga-like toxin-producing Escherichia coli. Supportive management is generally recommended. CASE REPORT A 58-year-old female with diarrhea-associated HUS developed delayed-onset severe neurological manifestations including coma, status epilepticus, and subcortical magnetic resonance imaging signal alterations. Rescue treatment with immunoglobulin (Ig)G depletion through immunoadsorption was followed by significant improvement in neurological and renal function. The patient recovered with only minimal sequelae. CONCLUSION: Delayed-onset neurological abnormalities may occur in diarrhea-associated HUS. Novel specific treatment options include IgG depletion through immunoadsorption. Severe clinical and imaging findings do not preclude a good outcome.