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Semin Thromb Hemost 2021; 47(01): 084-089
DOI: 10.1055/s-0040-1718870
Review Article

ABO Blood Group and Inhibitor Risk in Severe Hemophilia A Patients: A Study from the Italian Association of Hemophilia Centers

Massimo Franchini
1   Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy
,
Antonio Coppola
2   Regional Reference Center for Inherited Bleeding Disorders, University Hospital, Parma, Italy
,
Cristina Santoro
3   Department of Hematology, Policlinico Umberto I University Hospital, Rome, Italy
,
Raimondo De Cristofaro
4   Hemorrhagic and Thrombotic Diseases Service, Area of Hematology, Fondazione Policlinico Universitario “A. Gemelli,” IRCCS, Rome, Italy
,
Giovanni Barillari
5   Center for Hemorrhagic and Thrombotic Disorders, General and University Hospital, Udine, Italy
,
Paola Giordano
6   Pediatric Unit “B. Trambusti,” Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro,” Bari, Italy
,
Paolo Gresele
7   Section of Internal and Cardiovascular Medicine, Department of Medicine, University of Perugia, Perugia, Italy
,
Giuseppe Lassandro
6   Pediatric Unit “B. Trambusti,” Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro,” Bari, Italy
,
Mariasanta Napolitano
8   Hematology Unit, Department of Health Promotion, Mother and Child Care, Reference Regional Center for Thrombosis and Haemostasis, Internal Medicine and Medical Specialties, University of Palermo, Palermo, Italy
,
Giuseppe Lucchini
1   Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy
,
Matteo Luciani
9   Oncohematology Department, Pediatric Hospital Bambino Gesù, Rome, Italy
,
Antonietta Ferretti
3   Department of Hematology, Policlinico Umberto I University Hospital, Rome, Italy
,
Erminia Baldacci
3   Department of Hematology, Policlinico Umberto I University Hospital, Rome, Italy
,
Federica Riccardi
2   Regional Reference Center for Inherited Bleeding Disorders, University Hospital, Parma, Italy
,
Rita Carlotta Santoro
10   Center for Hemorrhagic and Thrombotic Disorders, “Pugliese-Ciaccio” Hospital, Catanzaro, Italy
,
Samantha Pasca
11   Hemophilia Center, University Hospital of Padua, Padua, Italy
,
Ezio Zanon
11   Hemophilia Center, University Hospital of Padua, Padua, Italy
,
Annarita Tagliaferri
2   Regional Reference Center for Inherited Bleeding Disorders, University Hospital, Parma, Italy
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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.

Keywords

ABO blood group - hemophilia - inhibitors - risk factors

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.




Publication History

Article published online:
01 February 2021

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