Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury.

ABSTRACT

Paroxysmal nocturnal hemoglobinuria is a rare form of intravascular hemolysis caused by an acquired deficiency of complement regulatory glycoproteins. In our case, a 53-year-old male presented with fatigue, discoloration of urine, and reduced urine output. Preliminary investigations showed severe anemia (3.7 g/dl) and hyperkalemia (7.6 mmol/L) in the setting of acute kidney injury, requiring urgent dialysis. Four units of packed cell volumes were transfused for the correction of anaemia. Following initial stabilization, flow cytometry and a fluorescein-labeled proaerolysin (FLAER) study showed a total deficiency of CD59 in 95.92% of granulocytes and a 97.14% deficiency in monocytes. A bone marrow biopsy showed erythroblast hyperplasia confirming the diagnosis of classical paroxysmal nocturnal hemoglobinuria. The patient was treated with steroids, androgens, and iron supplementation and made a complete recovery with a near-total resolution of his acute kidney injury. This paper aims to review the clinical features and investigations in order to focus on acute kidney injury as an outcome of paroxysmal nocturnal hemoglobinuria.