Blood Group O Does Not Increase the Risk of Inhibitors in Severe Haemophilia A: Data from the PedNet Study Group.

IF 3 2区医学 Q2 HEMATOLOGY

Haemophilia Pub Date : 2025-03-23 DOI:10.1111/hae.70035

Marloes de Kovel, Aimée-Claire van Haaster, Manuel Carcao, Susanna Ranta, Heidi Glosli, Georges E Rivard, Gili Kenet, Karin Kurnik, Christel Van Geet, Manuela Carvalho, Nadine G Andersson, Mutlu Kartal-Kaess, Rolf Ljung, H Marijke van den Berg

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引用次数: 0

Abstract

Introduction: Inhibitor development against factor VIII (FVIII) is the most common complication of hemophilia A replacement therapy. One of the variables considered to influence inhibitor development is the ABO blood group. Patients with blood group O have, on average, a 30%-40% lower endogenous von Willebrand factor (VWF) concentration. It has been postulated that VWF levels influence inhibitor development. The objective of this study was to investigate the inhibitor risk in patients with severe hemophilia A comparing those with blood group O with those with non-O blood groups.

Methods: The study population consisted of children with severe hemophilia A, born between 2000 and 2020, who reached 50 FVIII exposure days in the PedNet registry. Inhibitors were considered to be clinically relevant when at least two consecutive measurements were tested positive.

Results: Routine testing of blood groups varied between centres: Out of 1172 patients with severe hemophilia A, blood group status was known in 759 patients (65.8%). The relative risk of inhibitor development for blood group O in comparison to non-O was 1.04 (95% CI: 0.7-1.7).

Conclusion: In the PedNet cohort, blood group O did not increase the risk of inhibitors in previously untreated children with severe hemophilia A.

Trial registration: PedNet Registry; clinicaltrials.gov identifier: NCT02979119.

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来源期刊

Haemophilia 医学-血液学

CiteScore

6.50

自引率

28.20%

发文量

226

审稿时长

3-6 weeks

期刊介绍： Haemophilia is an international journal dedicated to the exchange of information regarding the comprehensive care of haemophilia. The Journal contains review articles, original scientific papers and case reports related to haemophilia care, with frequent supplements. Subjects covered include: clotting factor deficiencies, both inherited and acquired: haemophilia A, B, von Willebrand''s disease, deficiencies of factor V, VII, X and XI replacement therapy for clotting factor deficiencies component therapy in the developing world transfusion transmitted disease haemophilia care and paediatrics, orthopaedics, gynaecology and obstetrics nursing laboratory diagnosis carrier detection psycho-social concerns economic issues audit inherited platelet disorders.