Hamostaseologie 2019; 39(02): 195-202
DOI: 10.1055/s-0038-1673415
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Endothelial Function in Patients with Severe and Moderate Haemophilia A and B

Stephanie Böhmert
1   Division of Haemostaseology, Department of Internal Medicine II, University Hospital Frankfurt, Frankfurt, Germany
,
Ralf Schubert
2   Department for Children and Adolescents Medicine, University Hospital Frankfurt, Frankfurt, Germany
,
Stephan Fichtlscherer
3   Division of Cardiology, Department of Internal Medicine III, University Hospital Frankfurt, Frankfurt, Germany
,
Sonja Alesci*
4   IMD Blood Coagulation Centre, Bad Homburg, Germany
,
Wolfgang Miesbach*
1   Division of Haemostaseology, Department of Internal Medicine II, University Hospital Frankfurt, Frankfurt, Germany
› Author Affiliations
Further Information

Publication History

09 March 2018

13 August 2018

Publication Date:
09 October 2018 (online)

Abstract

The life expectancy of patients with haemophilia has increased and therefore the interest in age-related comorbidities has grown. The aim of this study was to determine whether haemophilia patients have a different endothelial function compared with the general population. A total of 26 patients with severe or moderate haemophilia A or B, 14 controls and 36 patients with coronary artery disease (CAD) were included in this study. Five markers of endothelial dysfunction (MOEDs) were determined. Moreover, the endothelial function was examined using the Itamar Endo-PAT, and the reactive hyperemia index (RHI) was calculated from the results. The MOEDs soluble intercellular adhesion molecule-1 (p = 0.0095) and interleukin-6 (p = 0.010) were significantly higher for patients with haemophilia compared with the control group. The presence of increased adhesion molecule levels and low-grade inflammation is suggestive of a decreased endothelial function. RHI is impaired in CAD patients (1.862), whereas haemophilia patients have an RHI of 1.958 in comparison with 2.112 in controls (p = 0.127). Therefore, laboratory and functional measurements imply a possible higher risk for CAD in haemophilia patients.

Zusammenfassung

Die Lebenserwartung der Hämophilie-Patienten und das Interesse an altersbedingten Begleiterkrankungen ist gestiegen. Die Fragestellung war, ob sich die endotheliale Funktion bei Hämophilie-Patienten und der Allgemeinbevölkerung unterscheiden. 26 Patienten mit mittelschwerer/schwerer Hämophilie A/B, 36 Patienten mit Koronarer Herzkrankheit (KHK) und 14 Normalpersonen wurden eingeschlossen. Fünf Marker für endotheliale Dysfunktion (MFED) wurden bestimmt. Die Endothelfunktion wurde mittels des Itamar Endo-PAT-Geräts gemessen und aus den Ergebnissen der Reaktive-Hyperämie-Index (RHI) berechnet. Bei den MFED zeigten sich signifikant höhere Werte für soluble intercellular adhesion molecule-1 (p = 0,0095*) und Interleukin-6 (p = 0,010*) bei Hämophilie-Patienten im Vergleich zur Kontrollgruppe. Das Vorhandensein von erhöhten Leveln von Adhäsionsmolekülen und geringgradiger Entzündung deuten auf eine verminderte endotheliale Funktion hin. Diese Tendenz spiegelt sich auch in den RHI-Ergebnissen wider (mittlerer RHI: KHK 1,862; Hämophilie 1,958; Kontrolle 2,112; p = 0,127). Laborchemische und funktionelle Untersuchungen deuten auf ein mögliches höheres Risiko für KHK bei Hämophilie-Patienten hin.

Authors' Contributions

S. B. collected the data, analysed and interpreted the data, drafted and wrote the manuscript; S. A. designed the study and reviewed the manuscript; R. S. helped with data collection, assisted with data analysis and reviewed the manuscript; S. F. reviewed the manuscript; W. M. assisted in writing the manuscript, revised and reviewed the manuscript. All authors approved the submitted and final version.


* Sonja Alesci and Wolfgang Miesbach are sharing last authorship.