A case of acute renal failure in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic hemolytic anemia characterized by persistent intravascular hemolysis. The kidneys are subject to acute failure or chronic injury in association with ischemia, intravascular hemolysis, and hemosiderin deposition; however the pathogenesis of renal failure has not been clearly defined. In Korea, there has been 1 case of acute renal failure with PNH demonstrated by MRI and a kidney biopsy. A 39-year-old female patient with PNH who received low dose oral prednisolone for 2 years developed oliguric acute renal failure after 2 days of fever and sore throat. The serum LDH level was elevated, and indirect bilirubinemia was noted. A renal biopsy showed features of acute tubular necrosis and hemosiderosis. Magnetic resonance imaging revealed characteristic features of renal hemosiderosis. There was no evidence of either renal vein thrombosis or cortical infarct. Her renal function became stabilized with hemodialysis and intravenous fluid, and she recovered 4 weeks later.