ABSTRACT

Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare clonal hematopoietic stem cell disorders that presents with pancytopenia findings, darkening of urine color and/ or venous thromboembolism. Determination of the level of CD55, CD59 by flow cytometry is the gold standard for diagnosis. Eculisumab reduces hemolysis and related complications in PNH’ s cases, by the inhibition of complement. Herein, we reported a case who admitted to our clinic with severe hemolysis, acute renal failure, diagnosed with PNH and treated by eculisumab.