PB0288 - The diagnostic value of thrombin generation in patients with bleeding disorders.

Sunday, June 23, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Amaury Monard

PhD candidate
Internal Medicine, Maastricht University Medical Center +, Maastricht, the Netherlands
Maastricht, Limburg, Netherlands

Co-Author(s)

ER

Eleonore Ravoet

Medical Doctor
Maastricht University Medical Center +, department of Internal medicine – hematology, Maastricht, the Netherlands
Maastricht, Limburg, Netherlands
CH

Caspar Hulleman

Medical Doctor
Maastricht University Medical Center +, department of Internal medicine – hematology, Maastricht, the Netherlands
Maastricht, Limburg, Netherlands
Rv

Rene van Oerle

researcher
MUMC
Maastricht, Limburg, Netherlands
Tv

Tom van de berg

Medical Doctor, hematologist in training
Maastricht University Medical Center +, department of Internal medicine – hematology, Maastricht, the Netherlands
Maastricht, Limburg, Netherlands
EB

Erik Beckers

Professor Hematology
Department of Internal medicing - Hematology, Maastricht University Medical Center +, Maastricht, the Netherlands
Maastricht, Limburg, Netherlands
Yvonne Henskens

Professor of the department of clinical chemistry
MUMC+
maastricht, Limburg, Netherlands
Floor Heubel-Moenen

MD, PhD
Department of Hematology, Maastricht University Medical Center, Maastricht, The Netherlands
Maastricht, Limburg, Netherlands

Background: The diagnostic work-up of patients with an increased bleeding tendency is time-consuming and often does not result in a clear diagnosis, as up to 40-70% of these patients is eventually classified as having a Bleeding Disorder of Unknown Cause (BDUC). This indicates that currently available laboratory hemostasis tests are not able to diagnose a large part of this patient population. The thrombin generation (TG) assays aim to study the hemostasis process more globally, possibly leading to new insights in the pathophysiological bleeding mechanisms in patients with an increased bleeding tendency.

Aims: To evaluate the diagnostic value of TG in patients with an increased bleeding tendency and to investigate the determinants of TG.

Methods: Patients referred to the hematologist for analysis of an increased bleeding tendency were included in the ProBe-AHP and the BEPA studies. Patients were divided into three groups; no bleeding disorder (NBD, n=45), Bleeding Disorder of Unknown Cause (BDUC, n=48) and a known bleeding disorder (BD, n=118). Extensive laboratory hemostasis testing was done and the Calibrated Automated Thrombogram (CAT-1) method with low concentrations of tissue factor was used.

Results: No statistically significant differences were seen in TGA parameters (lag time, ETP, Peak Height and Velocity Index) between the three patient groups. Subgroup analysis for each bleeding disorder showed statistically significant differences in lag time, ETP, peak height and velocity index for the von Willebrand’s disease, factor deficiency and hemophilia subgroups, when compared to the NBD-group (figure 1).

Conclusion(s): TG-parameters were significantly different between hemophilia patients and NBD-patients, but the BDUC-group showed no significant differences in TG-parameters compared to the NBD-group. These results indicate that our current TG setup is not able to further elucidate the bleeding mechanism in the BDUC-population. This could be explained by the large heterogeneity within the BDUC-population.