Management of pregnancy and delivery in congenital fibrinogen disorders: communication from the ISTH SSC Subcommittee on Factor XIII and Fibrinogen

Alessandro Casini; Rezan Abdul Kadir; Magy Abdelwahab; Marilyn J Manco-Johnson; Sanj Raut; Cecil Ross; Philippe de Moerloose; Cristina Santoro; Suchitra Acharya

doi:10.1016/j.jtha.2024.01.008

Management of pregnancy and delivery in congenital fibrinogen disorders: communication from the ISTH SSC Subcommittee on Factor XIII and Fibrinogen

J Thromb Haemost. 2024 May;22(5):1516-1521. doi: 10.1016/j.jtha.2024.01.008. Epub 2024 Jan 22.

Authors

Alessandro Casini¹, Rezan Abdul Kadir², Magy Abdelwahab³, Marilyn J Manco-Johnson⁴, Sanj Raut⁵, Cecil Ross⁶, Philippe de Moerloose⁷, Cristina Santoro⁸, Suchitra Acharya⁹

Affiliations

¹ Division of Angiology and Hemostasis, University Hospitals of Geneva, Geneva, Switzerland. Electronic address: alessandro.casini@hcuge.ch.
² Department of Obstetrics and Gynaecology, Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free London National Health Service Foundation Trust, London, UK; Institute for Women's Health, University College London, London, UK.
³ Pediatric Hematology Department, Cairo University Pediatric Hospital, Social and Preventive Medicine, Kasralainy Hospital, Cairo, Egypt.
⁴ Department of Pediatrics, Hemophilia and Thrombosis Center, University of Colorado, Anschutz Medical Campus, Aurora, Colorado, USA.
⁵ Haemostasias Section, Biotherapeutics Group, National Institute for Biological Standards and Control, Potters Bar, UK.
⁶ Department Medicine and Hematology, St. John's Medical College Hospital, Bangalore, Karnataka, India.
⁷ Faculty of Medicine, University of Geneva, Geneva, Switzerland.
⁸ Hematology, Hemophilia and Thrombosis Center, University Hospital Policlinico Umberto I, Rome, Italy.
⁹ Hemostasis and Thrombosis Center, Northwell Health, New Hyde Park, New York, USA.

PMID: 38266678
DOI: 10.1016/j.jtha.2024.01.008

Abstract

Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and/or qualitative fibrinogen deficiencies. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (ie, afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency for 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 International Society on Thrombosis and Haemostasis (ISTH) Scientific and Standardization Committee 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.

Keywords: afibrinogenemia; congenital; fibrinogen; fibrinogen deficiency; hypofibrinogenemia; pregnancy.

Publication types

Practice Guideline
Review

MeSH terms

Afibrinogenemia* / blood
Afibrinogenemia* / diagnosis
Afibrinogenemia* / therapy
Consensus
Delivery, Obstetric
Factor XIII / metabolism
Female
Fibrinogen* / metabolism
Fibrinogen* / therapeutic use
Humans
Pregnancy
Pregnancy Complications, Hematologic* / blood
Pregnancy Complications, Hematologic* / diagnosis
Pregnancy Complications, Hematologic* / therapy

Substances

Fibrinogen
Factor XIII