O型血不会增加严重血友病A抑制剂的风险：来自PedNet研究组的数据

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

摘要

针对因子VIII （FVIII）的抑制剂发展是血友病A替代治疗中最常见的并发症。ABO血型被认为是影响抑制剂发展的变量之一。O型血患者的内源性血管性血友病因子（VWF）浓度平均低30%-40%。据推测，VWF水平影响抑制剂的发育。本研究的目的是比较O型血和非O型血的严重血友病A患者抑制剂的风险。方法：研究人群包括2000年至2020年出生的严重血友病A患儿，在PedNet登记中达到50 FVIII暴露日。当至少连续两次测量结果为阳性时，抑制剂被认为具有临床相关性。结果：各中心之间的常规血型检测存在差异：在1172例严重A型血友病患者中，759例（65.8%）患者的血型状况已知。O型血患者与非O型血患者相比，抑制剂发展的相对风险为1.04 （95% CI: 0.7-1.7）。结论：在PedNet队列中，O型血并未增加先前未经治疗的严重血友病a患儿使用抑制剂的风险。clinicaltrials.gov识别码：NCT02979119。

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

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.