Management of Pregnancy and Delivery in Congenital Fibrinogen Disorders: Communication from the ISTH SSC Subcommittee on Factor XIII and Fibrinogen

Authors: Casini, A; Kadir, RA Abdelwahab, M; Manco-Johnson, MJ.; Raut, S; Ross, C; Moerloose, Philippe de; S, Cristina; Acharya, S.

Affiliations: Division of Angiology and Hemostasis, University Hospitals of Geneva, Geneva, Switzerland. Department of Obstetrics and Gynaecology; Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free London NHS Foundation Trust, London, United Kingdom; Institute for Women’s Health, University College London, London, United Kingdom. Pediatric Hematology Department, Cairo University Pediatric Hospital, Social and Preventive Medicine, Kasralainy Hospital, Cairo, Egypt. Department of Pediatrics, Hemophilia and Thrombosis Center, University of Colorado, Anschutz Medical Campus, Aurora, CO. Haemostasias Section, Biotherapeutics Group, National Institute for Biological Standards and Control, Potters Bar, UK. Department Medicine & Hematology, St. John’s Medical College Hospital, Bangalore, Karnataka, India. Faculty of Medicine, University of Geneva, Geneva, Switzerland. Hematology, Hemophilia and Thrombosis Center, University Hospital Policlinico Umberto I, Rome, Italy. Hemostasis and Thrombosis Center, Northwell Health, New Hyde Park, New York, USA.

Publication: Journal of thrombosis and haemostasis. 2024

ABSTRACT: Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and / or qualitative fibrinogen deficiency. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (i.e., afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency to miscarriages, bleeding, and thrombosis. Even though it is well established that management of such pregnancies requires a multidisciplinary approach involving specialists (hematologists and maternal / fetal medicine experts with expertise in the management of inherited bleeding disorders), specific guidelines are lacking. In this ISTH SSC communication, we aim to propose an expert consensus opinion with literature evidence where available on the strategy for management of pregnancy, delivery, and puerperium in CFDs.