Paroxysmal Nocturnal Haemoglobinuria (classic category)

Abstract

Paroxysmal nocturnal haemoglobinuria (PNH) is a rare disease. The prevalence of clinically significant PNH (i.e. classic PNH) plus patients with relatively large clones that arises in the setting of another marrow failure syndrome is likely in the order of less than 1 case per 200,000 population. Here we present a case of classic PNH who presented with acute kidney injury (AKI) requiring haemodialysis secondary to intravascular haemolysis and haemoglobin of 3.6 gm %. Her diagnosis was suggested by positive HAMS test and confirmed by Flow Cytometry. Patient recovered from AKI after starting danazol and her haemolysis was reduced but not controlled fully. Patient is awaiting treatment with either eculizumab or allogenic bone marrow transplantation