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

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...

Full description

Saved in:
Bibliographic Details
Published in:Scientific reports 2018-09, Vol.8 (1), p.13458-9, Article 13458
Main Authors: Alashkar, Ferras, Schemuth, Haemi Phaedra, Nensa, Felix, Göbel, Juliane, Vance, Colin, Forsting, Michael, Dührsen, Ulrich, Schlosser, Thomas Wilfried, Röth, Alexander
Format: Article
Language:English
Subjects:
Cerebral blood flow
Health risk assessment
Ischemia
Magnetic resonance imaging
Morbidity
Myocardial infarction
NMR
Nuclear magnetic resonance
Paroxysmal nocturnal hemoglobinuria
Patients
Thalamus
Thromboembolism
Thrombosis
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2045-2322
2045-2322
DOI:10.1038/s41598-018-31547-7