{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T01:04:48Z","timestamp":1760576688825,"version":"build-2065373602"},"reference-count":66,"publisher":"Georg Thieme Verlag KG","issue":"05","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hamostaseologie"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Congenital afibrinogenemia and hypofibrinogenemia are rare hereditary coagulation disorders characterized by the absence or deficiency of fibrinogen. These conditions pose unique challenges for women across their lifespan, including heavy menstrual bleeding (HMB), hemorrhagic ovarian cysts, complications during pregnancy and the postpartum period, as well as bleeding occurring later in life. HMB is frequent and adversely impacts quality of life, often necessitating hormonal therapy, antifibrinolytics, or fibrinogen replacement. Hemorrhagic ovarian cysts can result in life-threatening hemoperitoneum, requiring prompt intervention to manage bleeding and preserve ovarian function. Pregnancy in women with severe fibrinogen deficiencies carries a high risk of miscarriage, placental abruption, and postpartum hemorrhage. Multidisciplinary care, fibrinogen replacement, and vigilant monitoring are crucial to optimize maternal and fetal outcomes. Although understudied in this population, bleeding can occur later in their life, especially due to the increased incidence of gynecological pathologies. Tailored management strategies, including hormonal and surgical interventions, are essential. Despite recent advances in our understanding of these conditions, significant knowledge gaps persist regarding the prevalence, risk factors, and optimal management of specific complications. This review synthesizes current findings and provides practical recommendations to guide the care of young and ageing women with afibrinogenemia and hypofibrinogenemia. Further research is needed to refine treatment protocols and improve outcomes for this vulnerable population.<\/jats:p>","DOI":"10.1055\/a-2568-1255","type":"journal-article","created":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T19:34:37Z","timestamp":1748374477000},"page":"390-395","source":"Crossref","is-referenced-by-count":0,"title":["Management of Young and Ageing Women with Afibrinogenemia and Hypofibrinogenemia"],"prefix":"10.1055","volume":"45","author":[{"given":"Alessandro","family":"Casini","sequence":"additional","affiliation":[{"name":"Division of Angiology and Hemostasis, University Hospitals of Geneva, Geneva, Switzerland"},{"name":"Faculty of Medicine, University of Geneva, Geneva, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe","family":"de Moerloose","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Geneva, Geneva, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2025,5,27]]},"reference":[{"issue":"10","key":"ref1","doi-asserted-by":"crossref","first-page":"1712","DOI":"10.1182\/blood-2012-09-306639","article-title":"Mechanisms of fibrin polymerization and clinical implications","volume":"121","author":"J W Weisel","year":"2013","journal-title":"Blood"},{"issue":"08","key":"ref2","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1182\/blood.2024025712","article-title":"How I treat quantitative fibrinogen disorders?","volume":"145","author":"A Casini","year":"2025","journal-title":"Blood"},{"issue":"05","key":"ref3","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1182\/blood-2018-06-820738","article-title":"Treatment of rare factor deficiencies other than hemophilia","volume":"133","author":"M Menegatti","year":"2019","journal-title":"Blood"},{"issue":"09","key":"ref4","doi-asserted-by":"crossref","first-page":"1887","DOI":"10.1111\/jth.14216","article-title":"Diagnosis and classification of congenital fibrinogen disorders: communication from the SSC of the ISTH","volume":"16","author":"A Casini","year":"2018","journal-title":"J Thromb Haemost"},{"issue":"04","key":"ref5","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1111\/j.1538-7836.2012.04653.x","article-title":"Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders","volume":"10","author":"F Peyvandi","year":"2012","journal-title":"J Thromb Haemost"},{"issue":"06","key":"ref6","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1182\/bloodadvances.2023012186","article-title":"Congenital fibrinogen disorders: a retrospective clinical and genetic analysis of the Prospective Rare Bleeding Disorders Database","volume":"8","author":"S Mohsenian","year":"2024","journal-title":"Blood Adv"},{"issue":"02","key":"ref7","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1111\/hae.14738","article-title":"High incidence of intracranial haemorrhage in Egyptian children with congenital afibrinogenaemia","volume":"29","author":"M Abdelwahab","year":"2023","journal-title":"Haemophilia"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"100793","DOI":"10.1016\/j.blre.2020.100793","article-title":"Heterogeneity of congenital afibrinogenemia, from epidemiology to clinical consequences and management","volume":"48","author":"A Casini","year":"2021","journal-title":"Blood Rev"},{"issue":"08","key":"ref9","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1055\/s-0041-1742170","article-title":"Mutations accounting for congenital fibrinogen disorders: An update","volume":"48","author":"M Richard","year":"2022","journal-title":"Semin Thromb Hemost"},{"issue":"03","key":"ref10","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1111\/bjh.19039","article-title":"Congenital fibrinogen disorders: Strengthening genotype-phenotype correlations through novel genetic diagnostic tools","volume":"203","author":"R Ramanan","year":"2023","journal-title":"Br J Haematol"},{"issue":"04","key":"ref11","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1055\/s-0042-1758117","article-title":"Planning pregnancy and birth in women with inherited bleeding disorders","volume":"49","author":"A K Malinowski","year":"2023","journal-title":"Semin Thromb Hemost"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"1076029620912819","DOI":"10.1177\/1076029620912819","article-title":"Women with congenital hypofibrinogenemia\/afibrinogenemia: From birth to death","volume":"26","author":"Y Zhang","year":"2020","journal-title":"Clin Appl Thromb Hemost"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.thromres.2019.07.004","article-title":"Women with inherited bleeding disorders - 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