Abstract Number: PB0866
Meeting: ISTH 2020 Congress
Background: Hemophilic (HF) arthropathy is a major complication in hemophilia. Collagen IV, XV and XVIII are responsible for maintaining the integrity of the vessel wall in the joint. Following joint remodelling and damage, the short isoform of collagen type XVIII is degraded, releasing measurable fragments.
Aims: Our goal was to quantify the measurable fragments of the endothelial specific isoform of type XVIII collagen (COL-18N) in severe hemophilia A patients and to assess its relation to the clinical and radiological data as well as hemophilia joint health score (HJHS), functional independence score for hemophilia (FISH), and hemophilia quality of life.
Methods: This cross-sectional study included 50 severe hemophilia A patients recruited from the Pediatric Hematology / Oncology unit, Children’s Hospital, Ain Shams University, Cairo, Egypt. Quantification of serum collagen type XVIII was done by ELISA. Assessment of joint status clinically using FISH and HJHS scores and radiologically by X-rays and ultrasound was performed.
Results: Hemophilia A patients had significantly higher median collagen type XVIII levels compared to the control group. Inhibitor positive hemophilia A patients and inhibitor negative as well as hemophilia A patients on NSAID and those not had comparable collagen type XVIII levels. Patients with ≥2 target joints had significantly higher collagen type XVIII level compared to those with one or those without target joints(p=< 0.001). There were significant positive correlations between collagen type XVIII level and the total HJHS, hem QOL, the Ultrasound total score and annual bleeding rate (p=0.000, p=0.006, p=0.014, p=0.000).
Conclusions: Our results demonstrate the high level of serum collagen type XVIII in haemophilia A patients and argued its benefit as a potential marker for monitoring the development of hemophilic arthropathies and tailoring the optimal treatment to prevent further joint damage.
To cite this abstract in AMA style:Abdelhafez N, Abdel Gawad Tantawy A, Ahmad Safwat N, Anwar El Sayed El Seteha K. Endothelial Specific Isoform of Type XVIII Collagen (COL-18N): A Marker of Vascular Integrity in Hemophilic Arthropathy [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/endothelial-specific-isoform-of-type-xviii-collagen-col-18n-a-marker-of-vascular-integrity-in-hemophilic-arthropathy/. Accessed September 21, 2023.
« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/endothelial-specific-isoform-of-type-xviii-collagen-col-18n-a-marker-of-vascular-integrity-in-hemophilic-arthropathy/