PB1857 THE ROLE OF WHOLE‐BODY MAGNETIC RESONANCE IMAGING IN THE DETECTION OF VASCULAR EVENTS IN PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA.

Abstract

Background:Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by intravascular hemolysis, cytopenias, and recurrent thrombosis. Thrombotic events are just the main cause of morbidity and mortality. Therefore, it is essential to detect them early to treat them and avoid sequels.Aims:To analyze the diagnostic value of the whole‐body magnetic resonance imaging (WB‐MRI) in the detection of symptomatic and silent vascular events.Methods:Retrospective study in which a 1.5 Tesla whole‐body magnetic resonance (Philips Achieva, Philips Medical Systems, Best, the Netherlands) with Gadolinium contrast was performed along 2017 and 2018 to patients diagnosed of classical PNH in Autonomous Community of the Region of Murcia, Spain. The image findings were analyzed.Four patients were included (3 men and 1 woman) with median age of 54 years, diagnosed of either PNH (n = 2) and AA/PNH (n = 2). At the time of investigation, mean size clon in granulocytes measured by FLAER was high (90.7 %, range 75.6‐99.3 %) and mean LDH was 341.7 U/L (range 204‐465 U/L; normal range 135‐214 U/L). Only one patient had prior history of thrombosis (Budd‐Chiari syndrome). All patients were treated according to Spanish PNH guidelines, including the use of complement inhibitor Eculizumab.Results:WB‐MRI findings showed unknown bone infarctions in two (50%) patients (patient 1 and patient 3). In patient 1, symptomatic bone infarctions was diagnosed (both femoral condyle and proximal tibias). Patient 3 showed an asymptomatic area of bone edema in distal left radial bone.Summary/Conclusion:Although the sample from our study is very small, it seems that WB‐MRI could be useful to evaluate vascular events, like bone infarctions, even subclinical ones. Our findings are consistent with the only article published to date (Alaskar et al. Sci Rep. 2018; 8: 13458). Further studies are needed to test the effectiveness of WB‐MRI in the detection of vascular events and their relationship as a thrombotic event in PNH.imageimage