Intravascular hemolysis and resulting plasma-free hemoglobin elevation has been associated with acute kidney injury via several mechanisms. Proximal tubular injury due to iron compound deposition and nitric oxide sequestration with subsequent impairment of the microcirculation and hypoperfusion are specific adverse effects of intravascular hemolysis that may contribute to acute kidney injury. Therefore, removal of plasma-free hemoglobin and restoration of normal nitric oxide vasodilatory mechanisms may play a beneficial role in treating acute kidney injury secondary to acute intravascular hemolysis. We report herein the case of a 13-year-old patient with severe intravascular hemolysis and acute kidney injury and describe the role of plasma exchange with plasma replacement in his successful recovery.
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