Publication:

Paroxysmal nocturnal haemoglobinuria presented as acute kidney injury

Paroxysmal nocturnal hemoglobinuria (PNH) is rare disease, caused by acquired somatic mutations in PIG-A gene. Renal involvement in PNH varies from reversible acute kidney injury (AKI) to chronic irreversible damage.  We are presenting a case of PNH in young female who presented as AKI. Final diagnosis was made by flowcytometry which showed evidence of PNH clone based upon analysis of a variety of GPI linked antibodies (FLAER, CD59) on monocytes, granulocytes and RBCs. PNH presenting as recurrent acute renal failure is extremely rare. We reported this case to highlight a rare, but potentially reversible cause of acute renal failure. These types of cases need high index of suspicion. Early diagnosis and treatment will help in preventing repeated episodes of AKI and thus chronic kidney disease.