Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by
complement-mediated
intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of
infections due to
complement-associated defects subsequent to
CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic
ulcers on both legs, where the recognition of
pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent
AERolysin) flow cytometric analysis. He was subsequently started on
eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient's
leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of
necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella
pneumonia infection. Courses of broad-spectrum
antibiotics, surgical
debridement, and superficial
skin grafting were applied with successful effect during ongoing
eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening
infections like NF.