ISTH Congress Abstracts

Official abstracts site for the ISTH Congress

MENU 
  • Home
  • Congress Archive
    • ISTH 2022 Congress
    • ISTH 2021 Congress
    • ISTH 2020 Congress
  • Resources
  • Search

Evaluation of Primary Haemostasis in Children and Young Adults with Haemophilia

M. Kürschner1, J. Lohse1, F. Boiti1, O. Tiebel2, R. Knöfler1

1Department of Pediatric Haemostaseology, Faculty of Medicine, Technical University Dresden, Dresden, Germany, 2Department of Clinical Chemistry and Laboratory Medicine, Faculty of Medicine, Technical University Dresden, Dresden, Germany

Abstract Number: PB0482

Meeting: ISTH 2021 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Basic

Background: Patients with haemophilia present individual differences in bleeding tendency despite similar levels of the clotting factors VIII (FVIII:C) and IX (FIX:C). In this context the additional presence of hereditary coagulopathies such as thrombophilia, deficiencies of other coagulation factors and impairment of primary haemostasis is discussed in the literature.

Aims: Analyzing primary haemostasis parameters mainly in pediatric patients with haemophilia A and B in comparison to a pediatric control group.

Methods: Specimens from 38 haemophilia patients (33 with haemophilia A: severe n=24, moderate n=1, mild n=8 and 5 with haemophilia B: severe n=3, moderate n=1, mild n =1) with a median age of 11 years (range: 1 to 32 years) and 30 healthy controls (median 5.5 years ranging from 1 to 19 years) were evaluated. Platelet function was investigated by impedance aggregometry and luminometry in whole blood, by light transmission aggregometry and flow cytometry in platelet rich plasma (PRP). Furthermore, the PFA 100® closure times, factor VIII- and IX-activities, von Willebrand factor parameters, hemogram, and blood group were determined. Informed consent was obtained.

Results: Patients showed a significant elevated maximal aggregation induced by arachidonic acid (final concentration: 0.5 mM) and collagen (1 µg/ml) in whole blood. In PRP a significantly reduced ADP-induced (5 µM) maximal aggregation was detected in the haemophilia group. A reduced CD63 expression induced by ADP was significantly more often seen in the patient’s group (50%) compared to controls (22%). CD63 expression by TRAP and mepacrine release showed no differences between the two groups. Patients had significantly lower platelet counts (median: 266.500/µl) compared to controls (312.000/µl.) No significant differences between the groups were detected for all other parameters.

Conclusions: Our data demonstrate only minor and most likely clinically non-relevant differences in primary haemostasis between patients with haemophilia and healthy controls.
The study was supported by an unrestricted grant from Intersero®.

To cite this abstract in AMA style:

Kürschner M, Lohse J, Boiti F, Tiebel O, Knöfler R. Evaluation of Primary Haemostasis in Children and Young Adults with Haemophilia [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/evaluation-of-primary-haemostasis-in-children-and-young-adults-with-haemophilia/. Accessed June 25, 2022.

« Back to ISTH 2021 Congress

ISTH Congress Abstracts - https://abstracts.isth.org/abstract/evaluation-of-primary-haemostasis-in-children-and-young-adults-with-haemophilia/

Simple Search

Supported By:

Takeda logo

ISTH 2022 Congress site

Visit the official web site for the ISTH 2022 Virtual Congress »

  • Help & Support
  • About Us
  • Cookies & Privacy
  • Wiley Job Network
  • Terms & Conditions
  • Advertisers & Agents
Copyright © 2022 John Wiley & Sons, Inc. All Rights Reserved.
Wiley