A case of paroxysmal nocturnal hemoglobinuria with recurrent acute renal failure / 대한내과학회지

ABSTRACT

Paroxysmal nocturnal hemoglobinuria (PNH) is a acquired clonal disorder of the hematopoietic stem cells characterized by chronic intravascular hemolysis, venous thrombosis, deficient hematopoiesis. Kidney involvement is usually benign and secondary to chronic deposition of hemosiderin. But, acute renal failure may rarely occur in association with a hemolytic crisis or thrombotic complication. Hemolytic crisis is precipitated by nonspecific factors, such as infection, surgery and transfusion. A 69-year- old man was admitted to our hospital who developed acute renal failure after operation of anal fistula. He was diagnosed with PNH accompanied with deep vein thrombosis later. After being treated by hemodialysis and oral low dose steroid, he was discharged with full recovery of renal function. But, he frequently was developed acute renal failure with crisis of PNH despite taking low dose steroid. So, we report a case of PNH with recurrent acute renal failure without precipitating factors.