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

Massimo Franchini; Antonio Coppola; Cristina Santoro; Raimondo De Cristofaro; Giovanni Barillari; Paola Giordano; Paolo Gresele; Giuseppe Lassandro; Mariasanta Napolitano; Giuseppe Lucchini; Matteo Luciani; Antonietta Ferretti; Erminia Baldacci; Federica Riccardi; Rita Carlotta Santoro; Samantha Pasca; Ezio Zanon; Annarita Tagliaferri

doi:10.1055/s-0040-1718870

Seminars in Thrombosis and Hemostasis, Table of Contents

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

¹Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy

,

Antonio Coppola

²Regional Reference Center for Inherited Bleeding Disorders, University Hospital, Parma, Italy

,

Cristina Santoro

³Department of Hematology, Policlinico Umberto I University Hospital, Rome, Italy

,

Raimondo De Cristofaro

⁴Hemorrhagic and Thrombotic Diseases Service, Area of Hematology, Fondazione Policlinico Universitario “A. Gemelli,” IRCCS, Rome, Italy

,

Giovanni Barillari

⁵Center for Hemorrhagic and Thrombotic Disorders, General and University Hospital, Udine, Italy

,

Paola Giordano

⁶Pediatric Unit “B. Trambusti,” Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro,” Bari, Italy

,

Paolo Gresele

⁷Section of Internal and Cardiovascular Medicine, Department of Medicine, University of Perugia, Perugia, Italy

,

Giuseppe Lassandro

⁶Pediatric Unit “B. Trambusti,” Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro,” Bari, Italy

,

Mariasanta Napolitano

⁸Hematology 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

¹Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy

,

Matteo Luciani

⁹Oncohematology Department, Pediatric Hospital Bambino Gesù, Rome, Italy

,

Antonietta Ferretti

³Department of Hematology, Policlinico Umberto I University Hospital, Rome, Italy

,

Erminia Baldacci

³Department of Hematology, Policlinico Umberto I University Hospital, Rome, Italy

,

Federica Riccardi

²Regional Reference Center for Inherited Bleeding Disorders, University Hospital, Parma, Italy

,

Rita Carlotta Santoro

¹⁰Center for Hemorrhagic and Thrombotic Disorders, “Pugliese-Ciaccio” Hospital, Catanzaro, Italy

,

Samantha Pasca

¹¹Hemophilia Center, University Hospital of Padua, Padua, Italy

,

Ezio Zanon

¹¹Hemophilia Center, University Hospital of Padua, Padua, Italy

,

Annarita Tagliaferri

²Regional Reference Center for Inherited Bleeding Disorders, University Hospital, Parma, Italy

› Author Affiliations

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

Full Text

References

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