A mini-review on paroxysmal nocturnal hemoglobinuria and a case of eculizumab treatment of PNH in elderly MDS patient

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a systemic, acquired clonal hematological disorder which results from loss of complement-inhibiting proteins (CD55 and CD 59) from the blood cell surface. Therefore, such patients develop chronic complement-mediated intravascular hemolysis, with thromboses manifesting as most severe clinical complications. Recommendations of International PNH groups discern certain risk groups based on detection of a pathological cell clone, including patients with myelodysplastic syndrome (MDS). Here we describe detection of a clinically sound PNH clone in an elderly patient with MDS, as well as positive results of patho-genetic treatment with Eculizumab.