The Role of Whole-Body Magnetic Resonance Imaging (WB-MRI) in Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH)

Ferras Alashkar,Thomas Wilfried Schlosser,Michael Forsting,Felix Nensa,Alexander Röth,Ulrich Dührsen,Colin Vance,Haemi Phaedra Schemuth,Juliane Göbel

doi:10.1038/s41598-018-31547-7

Abstract

In PNH thromboembolic events (TEs) represent the leading cause of morbidity and mortality. Between Dec 2013 and Jan 2016 37 PNH patients (pts) (23 PNH, 14 AA/PNH; 51% (19/37) females; median age 44 years, median D-dimer levels 0.22 mg/l) were examined with a whole-body magnetic resonance imaging (WB-MRI) scan at 1.5 T to detect TEs. Pts were treated according to German PNH guidelines, including eculizumab therapy. 64% (24/37) of the pts had no documented TEs prior to observation. Two pts had suspected TEs in their clinical history. 29% of the pts (11/37) had a known history of venous thromboses (deep venous thrombosis (DVT) (5/11), portal venous thrombosis (PVT) (4/11), vena caval thrombosis (VCT) (2/11). A myocardial infarction was reported in one pt, and two had a cerebral venous sinus thrombosis (CVST) or a thalamic infarction. Six pts (16%) had at least two prior TEs. In pts with prior TEs no progression of the existing TEs was observed. In pts on eculizumab and prior TEs as well as treatment-naïve pts silent bone and renal infarctions were detected. Furthermore, a clinically non-critical arterial occlusion was identified. WB-MRI scans present a novel, non-invasive method to assess the complete vascular status of PNH pts and allow the detection of previously undiagnosed vascular complications, affecting treatment indications and regimens.

Highlights

Treatment[10,11]
The diagnostic examination is dependent on the clinical symptoms of the patient, e.g. a patient presenting with unexplained abdominal pain and known PNH would be examined with an abdominal CT or MRI scan only
Between Dec. 2013 and Jan. 2016 clinically indicated whole-body magnetic resonance imaging (WB-MRI) scans were performed in 37 patients (51% (19/37) females; median age 44 years with either PNH (n = 23) or AA/PNH-syndrome and a detectable PNH clone (n = 14) of the currently 207 patients enrolled in the prospective, observational, non-interventional PNH-Registry of the University Hospital of Duisburg-Essen

Summary

Introduction

Treatment[10,11]. prophylactic anticoagulation still remains a controversial subject in these patients, especially in non-eculizumab-treated patients. Given that the rates of silent thromboses in PNH are likely underestimated[14], the importance of a prompt and accurate diagnosis of thrombotic events by high sensitive imagining is crucial and highly implicated in PNH, influencing the clinical outcome, mortality rates, and the need for treatment. The diagnostic examination is dependent on the clinical symptoms of the patient, e.g. a patient presenting with unexplained abdominal pain and known PNH would be examined with an abdominal CT or MRI scan only. Combined arterial and venous whole-body MR-angiography has been proven feasible at 1.5 T in patients with thromboembolic diseases to detect arterial and venous TEs15. The aim of this study was to examine the feasibility of WB-MRI to detect and monitor clinically apparent as well as silent TEs in PNH patients

Objectives

Methods

Results

Conclusion