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DOI: 10.1055/s-0040-1718870
ABO Blood Group and Inhibitor Risk in Severe Hemophilia A Patients: A Study from the Italian Association of Hemophilia Centers
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Abstract
Considering the profound influence exerted by the ABO blood group system on hemostasis, mainly through the von Willebrand factor and factor VIII (FVIII) complex, we have conducted a study evaluating the possible role of blood type on the risk of inhibitor development in hemophilia A. A total of 287 consecutive Caucasian patients with severe hemophilia A (202 without FVIII inhibitors and 85 with FVIII inhibitors) followed at seven Italian Hemophilia Treatment Centers belonging to the Italian Association of Hemophilia Centers (AICE) were included in the study. A higher prevalence of O blood group was detected in patients without inhibitors as compared in inhibitor patients (55 vs. 30.6%; p < 0.001). Among the other variables analyzed (age, F8 mutation, type and intensity of treatment and treatment regimen), F8 mutation class (high-risk vs. low-risk), and treatment regimen (on-demand vs. prophylaxis) were significantly correlated with inhibitor development. However, on a multivariate analysis, only the effects of F8 mutation and ABO blood type were independent of other covariates, being that non-O blood type is associated with a 2.89-fold increased risk of inhibitor development. In conclusion, our study supports the protective effect of O blood type on inhibitor risk in severely affected hemophilia A patients.
Authors' Contributions
M.F. designed the study and coordinated its implementation in the network of AICE centers and performed data collection, analysis, and interpretation, together with A.C. and A.T. C.S., R.D.C., G.B, P.G., P.Gr., G.L., M.N., G.Lu., M.L., A.F., R.C.S., S.P., and E.Z. enrolled patients and collected their data. F.R. contributed to the analysis of F8 gene mutations. M.F. and A.C. wrote the first draft of the manuscript. All the coauthors revised the manuscript and approved the final version.
Publikationsverlauf
Artikel online veröffentlicht:
01. Februar 2021
© 2021. Thieme. All rights reserved.
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