Hemolytic-uremic syndrome pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Pathophysiology

Molecular Biology

• Platelet-rich thrombi in affected organs (unclear etiology of tissue specificity CD36)
• vWF (endothelially synthesized) -> ULvWf multimers -> shear stress unfolds and causes massive platelet aggregation

• Normally, UlvWf digested by metalloprotease to “normal” size vWf multimers
• Familial forms of TTP lack metalloprotease activity
• Acquired forms of TTP have IgG antibody, which reduce metalloprotease activity during flares

Metalloprotease activity appears normal in HUS

• In HUS, and in cases of TTP without decreased metalloprotease activity, other etiologies of platelet activation have been proposed:

• Endothelial injury (esp. drug induced)
• Toxins (i.e. Shiga toxin)
• PAI – 1
• Other genetic factors (Factor H, Factor I deficiencies, complement derangements).