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Hemolytic uremic syndrome: past and present

EMOLYTIC uremic syndrome (HUS) consists of nonimmune hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The characteristic lesion, thrombotic microangiopathy, consists of vessel wall thickening, with swelling and detachment of endothelial cells from the basement membrane and accumulation of fluffy material in the subendothelium. 1 HUS usually manifests as an acute disease, with gastrointestinal or respiratory tract prodromes, that spontaneously recovers without sequelae in 80% to 90% of cases (ie, most cases of childhood HUS) or after plasma infusion or exchange (ie, adult or severe forms of HUS). Environmental factors, drugs, or infective agents such as verotoxin-producing Escherichia coli trigger typical HUS; systemic immune disorders or cancer also may cause the disease. 2 These forms of HUS may subside when the underlying condition has been treated or removed. Rare forms occur and are often familial, with both autosomal recessive and dominant modes of inheritance.