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Thrombin Generation by Genesia in Patients with Haemophilia A and Inhibitors during Immune Tolerance Induction

T. Szanto1,2, E. Lehtinen1, M. Lemponen2, R. Lassila1,2

1Unit of Coagulation Disorders, Department of Hematology, Helsinki University Hospital Comprehensive Cancer Center, University of Helsinki, Helsinki, Finland, 2Research Program in Systems Oncology in Faculty of Medicine, University of Helsinki, Helsinki, Finland

Abstract Number: PB0465

Meeting: ISTH 2021 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Basic

Background: Inhibitor formation complicates hemophilia treatment and requires immune tolerance induction (ITI) to get rid of the high-titer inhibitors (> 5 BU/ml).

Aims: To assess the hemostatic response to FVIII replacement during ITI by studying thrombin generation (TG) related to traditional FVIII and inhibitor levels.

Methods: Seven hemophilia A patients undergoing ITI were studied. FVIII product was plasma-derived in one and recombinant in 6 patients with the dosing 100 IU/kg od. Patients underwent a laboratory assessment after 72-h wash-out period followed by a prospective observation of 12 months and a maximum of 36 months. TG by Genesia/STG-BleedScreen and inhibitor titers (BU/ml) by Nijmegen Bethesda assay were measured. FVIII and TG before and after FVIII infusions were periodically repeated during follow-up (at week 3, and at 3, 6 and 12 months).  ABR (annual bleeding rate) and QoL (quality of life) were assessed.

Results: During the ITI, half of the patients achieved complete response within 6 months, one within 36 months, while two other patients achieved partial response. The peak inhibitor titer during ITI varied between 0.6 to 717 BU/mL (median 34). Genesia detected the hemostatic response to FVIII and ITI outcome. Post-infusion FVIII levels (30 min) correlated positively with ETP at week 3 (FVIII:C: r= 0.9 and p= 0.02; FVIII chromogenic: r=0.9 and p= 0.04), and both with peak thrombin (FVIII:C: r= 0.8 and p= 0.04; FVIII chromogenic: r=0.9 and p= 0.02) and ETP (FVIII:C: r= 0.9 and p= 0.03; FVIII chromogenic: r=0.8 and p= 0.04) at 6 months. In contrast, inhibitor titers correlated negatively with post-infusion peak height (r= -0.9 and p=0.02) and ETP (r=-0.9 and p=0.003) at 3 months, and lag time at 6 and 12 months (r=-0.9, p=0.03 and r=-0.9, p=0.01). ABR and QoL improved.

Conclusions: TG by Genesia distinguished different responses with a high sensitivity in inhibitor patients undergoing FVIII replacement.

To cite this abstract in AMA style:

Szanto T, Lehtinen E, Lemponen M, Lassila R. Thrombin Generation by Genesia in Patients with Haemophilia A and Inhibitors during Immune Tolerance Induction [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/thrombin-generation-by-genesia-in-patients-with-haemophilia-a-and-inhibitors-during-immune-tolerance-induction/. Accessed November 29, 2023.

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