Volltextdatei(en) vorhanden
Titel: Einfluss der Methode zur Bestimmung der von Willebrand-Faktor-Aktivität auf die klinische Diagnostik des von Willebrand-Syndroms : eine retrospektive Analyse
Sonstige Titel: Retrospective comparison of two activity assays in the diagnosis of von Willebrand disease
Sprache: Deutsch
Autor*in: Heicks, Anne-Kathrin
Schlagwörter: VWS; von willebrand syndrom; VWF-Aktivität; VWF:Ac; VWF:RCo; VWD; von Willebrand disease; von Willebrand factor activity assay; GPIb binding assay
Erscheinungsdatum: 2015
Tag der mündlichen Prüfung: 2016-01-08
Zusammenfassung: 
Background: The diagnosis of VWD depends on clinical symptoms and laboratory findings. In this regard, a von Willebrand factor (VWF) activity assay is considered mandatory for the diagnostic work-up of patients with suspected inherited bleeding disorders. In September 2011, the VWF ristocetin cofactor assay (VWF:RCo) was replaced by the fully automated INNOVANCE® GPIb binding assay (VWF:Ac) at our institution. We retrospectively analyzed the effect of this methodological transition on the frequency and pattern of VWD diagnosis at our outpatient clinic.

Methods: All patients presenting with a presumed bleeding disorder during the year before (cohort VWF:RCo) and after introduction of the VWF:Ac assay (cohort VWF:Ac) were included in the analysis. Clinical data, including final diagnoses, and laboratory findings were extracted from electronic patient files.

Results: The VWF:RCo and VWF:Ac cohorts comprised 322 and 298 patients, respectively. In 10 patients, both assays were performed in parallel. Demographic patient characteristics were similar between the two groups (mean age, 50 ± 18 years; females, 70%). A decreased (i.e. below the normal reference range) VWF activity was more frequently detected in the VWF:RCo than in the VWF:Ac cohort (29% vs. 22%; P=0.04). Of the patients with decreased VWF activity, 22% (VWF:RCo) and 8% (VWF:Ac) had normal PFA-100® closure times (P=0.02), indicating regular VWF function under high shear conditions. VWF:Ac (r=0.92) correlated more closely with VWF collagen binding capacity (VWF:CB) than VWF:RCo (r=0.79). In the 10 patients with parallel measurements, results were significantly higher using the VWF:Ac (132 ± 50%) than the VWF:RCo assay (114 ± 41%; P=0.03). Importantly, in two of the ten patients with decreased VWF:RCo, but normal VWF:Ac, VWD was ruled out on the basis of repeated testing and clinical assessment. Likewise, in the VWF:Ac cohort, "possible VWD" was less frequently diagnosed (19% vs. 24%) and VWD was more often ruled out (65% vs. 59%) than in the VWF:RCo cohort. Consistently, the VWF:Ac assay had improved specificity (85% vs. 76%) and positive predictive value (44% vs. 33%) for the diagnosis of VWD than the VWF:RCo assay.

Conclusion: in this retrospective analysis of "real-world" patients presenting to an outpatient clinic for the diagnostic work-up of a presumed bleeding disorder, the VWF:Ac assay was associated with an improved diagnostic accuracy when compared to the VWF:RCo assay.
URL: https://ediss.sub.uni-hamburg.de/handle/ediss/6634
URN: urn:nbn:de:gbv:18-77654
Dokumenttyp: Dissertation
Betreuer*in: Langer, Florian (PD Dr.)
Enthalten in den Sammlungen:Elektronische Dissertationen und Habilitationen

Dateien zu dieser Ressource:
Datei Beschreibung GrößeFormat  
Dissertation.pdf451.7 kBAdobe PDFÖffnen/Anzeigen
Zur Langanzeige

Diese Publikation steht in elektronischer Form im Internet bereit und kann gelesen werden. Über den freien Zugang hinaus wurden durch die Urheberin / den Urheber keine weiteren Rechte eingeräumt. Nutzungshandlungen (wie zum Beispiel der Download, das Bearbeiten, das Weiterverbreiten) sind daher nur im Rahmen der gesetzlichen Erlaubnisse des Urheberrechtsgesetzes (UrhG) erlaubt. Dies gilt für die Publikation sowie für ihre einzelnen Bestandteile, soweit nichts Anderes ausgewiesen ist.

Info

Seitenansichten

51
Letzte Woche
Letzten Monat
geprüft am 09.05.2021

Download(s)

107
Letzte Woche
Letzten Monat
geprüft am 09.05.2021
Werkzeuge

Google ScholarTM

Prüfe