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Title: Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies
Authors: M. Napolitano
M. N.D. Di Minno
A. Batorova
A. Dolce
M. Giansily-Blaizot
J. Ingerslev
J. F. Schved
G. Auerswald
G. Kenet
M. Karimi
T. Shamsi
A. Ruiz de Sáez
R. Dolatkhah
A. Chuansumrit
M. A. Bertrand
G. Mariani
Universita degli Studi di Palermo
Università degli Studi di Napoli Federico II
University Hospital in Bratislava
Istituto Nazionale Di Statistica, Rome
CHU Montpellier
Skejby Sygehus, Aarhus University Hospital
Klinikum Bremen-Mitte
Chaim Sheba Medical Center Israel
Tel Aviv University, Sackler Faculty of Medicine
Shiraz University of Medical Sciences
National Institute of Blood Disease and Bone Marrow Transplantation
Banco Central de Venezuela
Tabriz University of Medical Sciences
Mahidol University
CRTH de Besançon
University of Westminster
Keywords: Medicine
Issue Date: 1-Sep-2016
Citation: Haemophilia. Vol.22, No.5 (2016), 752-759
Abstract: © 2016 John Wiley & Sons Ltd Introduction: A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency. Aim: Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. Methods: A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. Results: In our cohort (N = 449; 215 male, 234 female), the higher prevalence of mucocutaneous bleeds in females strongly predicted ensuing gynaecological bleeding (hazard ratio = 12.8, 95% CI 1.68–97.6, P = 0.014). Menorrhagia was the most prevalent type of bleeding (46.4% of patients), and was the presentation symptom in 12% of cases. Replacement therapies administered were also analysed. For surgical procedures (n = 50), a receiver operator characteristic analysis showed that the minimal first dose of rFVIIa to avoid postsurgical bleeding during the first 24 hours was 22 μg kg−1, and no less than two administrations. Prophylaxis was reported in 25 women with excellent or effective outcomes when performed with a total weekly rFVIIa dose of 90 μg kg−1(divided as three doses). Conclusion: Women with FVII deficiency have a bleeding disorder mainly characterized by mucocutaneous bleeds, which predicts an increased risk of ensuing gynaecological bleeding. Systematic replacement therapy or long-term prophylaxis with rFVIIa may reduce the impact of menorrhagia on the reproductive system, iron loss and may avoid unnecessary hysterectomies.
ISSN: 13652516
Appears in Collections:Scopus 2016-2017

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