Gynecological and obstetric outcome in the French cohort of women with factor XIII deficiency.

Autor: Rugeri L; Hospices Civils de Lyon, Unité Hémostase Clinique, Hôpital Louis Pradel, Hospices Civils de Lyon, Bron, France. Electronic address: lucia.rugeri@chu-lyon.fr., Martinaud C; Department of Medical Biology, Military Medical Center Percy, Clamart, France., Beurrier P; Centre de Traitement de l'Hémophilie, CHU Angers, Angers, France., Borg Y; Unité Hémostase et Centre Régional de Traitement des maladies Hémorragiques, Institut de Biologie Clinique, Hôpital Charles Nicolle, Rouen, France., Chambost H; Centre for Bleeding Disorders, Pediatric Haematology Oncology Department, University Hospital La Timone, AP-HM, and Aix Marseille Univ, INSERM, INRA, C2VN, Marseille, France., Chirila-Hetsch M; Service d'Hématologie, Hôpital de Valence, Valence, France., Desprez D; Centre de Traitement de l'Hémophilie, CHU Strasbourg, Strasbourg, France., Harroche A; Hemophilia Care Centre, Hematology Unit, Hôpital Universitaire Necker Enfants Malades, Paris, France., Milien V; FranceCoag, University Hospital La Timone, AP-HM, Marseille, France., Pan-Petesch B; Unité Hémostase Service hématologie, CHU Brest, Brest, France., Meunier S; Hospices Civils de Lyon, Unité Hémostase Clinique, Hôpital Louis Pradel, Hospices Civils de Lyon, Bron, France.
Jazyk: angličtina
Zdroj: Thrombosis research [Thromb Res] 2020 Jul; Vol. 191, pp. 22-25. Date of Electronic Publication: 2020 Apr 21.
DOI: 10.1016/j.thromres.2020.04.010
Abstrakt: Introduction: Congenital factor XIII deficiency is a very rare bleeding disorder affecting 33 patients in France. Besides its role in fibrin clot stabilization, factor XIII is involved in placental attachment. Fetal miscarriages represent a frequent and concerning issue for these patients. The aim of the present study was to describe clinical characteristics of women presenting severe congenital FXIII deficiency in France, to focus on gynecological and obstetrical events, and to report the management of these rare situations.
Methods: We conducted a retrospective study in the French Hemophilia Comprehensive Care and Clinical Hemostasis Centers. Women between 15 and 65 years with factor XIII activity <10 IU dL -1 were included. Biological, clinical and therapeutic events that occurred to these patients during their gynecological and obstetrical period were recorded.
Results: Among 31 centers, eleven patients were included. The median age at diagnosis was 1.5 years (range: 0-35), and at inclusion it was 30 years (range: 15-63). Fetal miscarriage was the primary manifestations in 2 (18%) patients, the remaining were diagnosed during hemorrhage. Menorrhagias were reported by 2 women (27%), 13 pregnancies were reported by 9 women including one abortion. Every pregnancy was conducted under factor XIII substitution, no hemorrhagic episode was reported. Four patients (36%) experienced at least one fetal miscarriage with a total amount of 30 miscarriages with 6 occurring during substitution.
Conclusion: Altogether, our data confirmed the high incidence of miscarriage in women with factor XIII deficiency. Good outcome of pregnancies required prophylaxis in accordance with international guidelines.
Competing Interests: Declaration of competing interest All authors declare no conflicts of interest.
(Copyright © 2020 The Authors. Published by Elsevier Ltd.. All rights reserved.)
Databáze: MEDLINE
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