Hemolytic Crisis and Acute Kidney Injury in Patient with Paroxysmal Nocturnal Hemoglobinuria in Korea : Case Report and Review of Literature / 대한신장학회지

ABSTRACT

Paroxysmal nocturnal hemoglobinuria (PNH) is an 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. However, acute kidney injury 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 35-year-old woman, who developed hemolytic crisis and acute kidney injury was admitted to our hospital presenting with acute gastroenteritis. After being treated by hemodialysis and oral low dose steroid, she was discharged with recovered renal function. Renal biopsy demonstrated acute tubular necrosis with considerable hemosiderin deposition without evidence of vascular thrombosis. A review of Korean cases showed that most of the cases featured severe renal dysfunction to such an extent to require a hemodialysis although there were no definite etiologies other than the deposition of blood iron due to massive hemolysis unlike the foreign cases. It also showed that the disease duration was longer. It can therefore be inferred that the early diagnosis and active treatment will be mandatory for the treatment of Korean patients with PNH. We reported a case of PNH with acute kidney injury and hemolytic crisis and documented by renal biopsy with review of Korean literature.