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The Thrombin Generation Test represents a better tool than Factor VIII levels to assess the risk of bleedings in patients with hemophilia A

1HUCA, oviedo, Asturias, Spain, 2Department of Hematology, Central University Hospital of Asturias, Oviedo, Asturias, Spain, 3University of Oviedo - ISPA, Oviedo, Asturias, Spain, 4HOSPITAL UNIVERSITARIO CENTRAL DE ASTURIAS, Oviedo, Asturias, Spain, 5Department of Internal Medicine, Central University Hospital of Asturias, Oviedo, Asturias, Spain, 6Department of Pharmacology, University of Navarra, Pamplona, Navarra, Spain, 7Haemostasis, Thrombosis, Arteriosclerosis and Vascular Biology Research Group, Medical Research Institute Hospital La Fe, Valencia, Comunidad Valenciana, Spain, 8Medical Research Institute Hospital La Fe, Valencia, Comunidad Valenciana, Spain

Abstract Number: PB1143

Meeting: ISTH 2022 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Hemophilia A (HA) is a rare bleeding disorder caused by factor VIII (FVIII) deficiency due to mutations in the F8 gene. Disease severity inversely correlates with plasma FVIII (FVIII:C) concentration. Unaccountably, a subgroup of severe HA patients scarcely bleed and do not require prophylactic FVIII administration, while moderate HA patients bleed frequently, despite their FVIII:C levels.

Aims: To evaluate the capacity of the ST Genesia® Thrombin Generation System (TGT) to ascertain the hemostatic status of the entire cohort of HA outpatients, treated and non- bleeding, in Asturias (Spain) and to compare it with FVIII performance.

Methods: We recruited 55 HA patients (18 severe, 7 moderate and 30 mild; FVIII:C < 40%) following treatment and without inhibitors, and 25 healthy controls. HA patients were monitored assessing FVIII activity (and vWF levels) at diagnosis and follow-up, and screened for F8 mutations. The TGT BleedScreen was evaluated during follow-up.

Results: Patients with severe/moderate HA had lower FVIII:C at diagnosis and a higher annual bleeding rate (ABR) than mild HA patients (P < 0.001) (Table 1). Importantly, on the day of the TGT and despite having lower FVIII:C, severe/moderate patients had a similar thrombin potential than mild patients, reflecting better the non-bleeding phase of our patient cohort (Figure 1). Additionally, a correlation of the F8 mutation type was observed with HA severity, ABR and hemostatic status.

Conclusion(s): The hemostatic status of HA patients measured with ST Genesia® correlates more precisely with their bleeding risk than classical FVIII:C. The F8 mutation type may indicate a worse prognosis, higher ABR and worse hemostatic status. The TGT may represent a more suitable tool than classical FVIII:C determination to identify HA patients that may require a closer follow-up and a tailored therapeutic adjustment in high-risk situations. MINECO (RYC-2013-12587), MICIU (SAF2017-85489-P), Roche (SP200221001), GVA (ACIF/2017/138) and Sociedad Española de Trombosis y Hemostasia.

Table 1

Table 1

Figure 1

Figure 1

To cite this abstract in AMA style:

Bernardo A, Soto I, Gutiérrez L, Caro A, Martínez-Carballeira D, Corte Buelga J, Vázquez S, Palomo-Antequera C, Andreu A, Fernández-Pardo A, Oto J, Medina P. The Thrombin Generation Test represents a better tool than Factor VIII levels to assess the risk of bleedings in patients with hemophilia A [abstract]. https://abstracts.isth.org/abstract/the-thrombin-generation-test-represents-a-better-tool-than-factor-viii-levels-to-assess-the-risk-of-bleedings-in-patients-with-hemophilia-a/. Accessed September 22, 2023.

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