Paroxysmal nocturnal hemoglobinuria and eculizumab

Abstract

Doi:10.3324/haematol.2009.017848 *This paper is dedicated to the dearest memory of Bruno Rotoli: for us and for many others a trusted colleague, inspiring mentor, and long-time friend. (Related Original Articles on pages 567 and 574) Paroxysmal nocturnal hemoglobinuria (PNH) is enoughof a tongue-twister for patients and doctors alike and weall teach the students that PNH is a disorder character-ized by the triad of intravascular hemolysis, venous thrombo-sis and cytopenias.1-3 But in this journal, founded by Adolfo Ferrata in the twenties, it seems appropriate to remember that, in the Italian literature, the full name coined for PNH in 1928 by Ettore Marchiafava was even more articulate: parox-ysmal nocturnal haemoglobinuria with perpetual haemosiderinuria.4 The last qualification is not trivial, because even today the accesses of hemoglobinuria, most distressing for the patient, may tend to obscure the fact that in PNH hemolysis goes on all the time: macroscopic hemoglobinuria merely reflects it