Case Report: Laboratory detection of a thrombotic tendency in a family with hypodysfibrinogenemia and a novel

case report congenital fibrinogen disorders diagnosis hypodysfibrinogenemia phenotype

Journal

Frontiers in cardiovascular medicine
ISSN: 2297-055X
Titre abrégé: Front Cardiovasc Med
Pays: Switzerland
ID NLM: 101653388

Informations de publication

Date de publication:
2024
Historique:
received: 30 08 2024
accepted: 26 09 2024
medline: 30 10 2024
pubmed: 30 10 2024
entrez: 30 10 2024
Statut: epublish

Résumé

Hypodysfibrinogenemia is a rare congenital fibrinogen disorder (CFD) which may induce thrombotic and bleeding events. Therefore, patient management needs careful evaluation. Routine coagulation tests are inadequate to predict the clinical phenotype. A 60-year-old woman with both bleeding and thrombotic complications and her two daughters were referred to our center for genotypic and phenotypic analysis of a CFD. Conventional laboratory results led to the diagnosis of hypodysfibrinogenemia in all three subjects. They all carried the same heterozygous c.1124A>G mutation in We report on a family with hypodysfibrinogenemia and a novel

Identifiants

pubmed: 39473893
doi: 10.3389/fcvm.2024.1488602
pmc: PMC11518780
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Pagination

1488602

Informations de copyright

© 2024 Monard, Castoldi, De Simone, Wichapong, van Duijl, van den Biggelaar, Spada, van Doorn, Hellenbrand, van der Meijden, Swieringa, Stork, ten Cate, Beckers, Heubel-Moenen and Henskens.

Déclaration de conflit d'intérêts

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Amaury Monard (A)

Department of Internal Medicine-Hematology, Maastricht University Medical Centre+, Maastricht, Netherlands.
CARIM, School for Cardiovascular Disease, Maastricht University, Maastricht, Netherlands.

Elisabetta Castoldi (E)

Department of Biochemistry, CARIM, Maastricht University, Maastricht, Netherlands.

Ilaria De Simone (I)

Department of Biochemistry, CARIM, Maastricht University, Maastricht, Netherlands.

Kanin Wichapong (K)

Department of Biochemistry, CARIM, Maastricht University, Maastricht, Netherlands.

Tirsa van Duijl (T)

Department of Molecular Hematology, Sanquin Research, Amsterdam, Netherlands.

Maartje van den Biggelaar (M)

Department of Molecular Hematology, Sanquin Research, Amsterdam, Netherlands.

Stefano Spada (S)

Department of Biochemistry, CARIM, Maastricht University, Maastricht, Netherlands.
Centre for Thrombosis and Hemostasis (CTH), University Medical Centre Mainz, Mainz, Germany.

William van Doorn (W)

Central Diagnostic Laboratory, Maastricht University Medical Centre+, Maastricht, Netherlands.

Dave Hellenbrand (D)

Central Diagnostic Laboratory, Maastricht University Medical Centre+, Maastricht, Netherlands.

Paola van der Meijden (P)

CARIM, School for Cardiovascular Disease, Maastricht University, Maastricht, Netherlands.
Thrombosis Expertise Center, Heart+ Vascular Center, Maastricht University Medical Center+, Maastricht, Netherlands.

Frauke Swieringa (F)

Department of Biochemistry, CARIM, Maastricht University, Maastricht, Netherlands.

Alexander Stork (A)

Department of Internal Medicine, Anna Hospital, Geldrop, Netherlands.

Hugo Ten Cate (H)

Department of Biochemistry, CARIM, Maastricht University, Maastricht, Netherlands.
Department of Internal Medicine, Maastricht University, Maastricht, Netherlands.

Erik Beckers (E)

Department of Internal Medicine-Hematology, Maastricht University Medical Centre+, Maastricht, Netherlands.

Floor Heubel-Moenen (F)

Department of Internal Medicine-Hematology, Maastricht University Medical Centre+, Maastricht, Netherlands.

Yvonne Henskens (Y)

CARIM, School for Cardiovascular Disease, Maastricht University, Maastricht, Netherlands.
Department of Molecular Hematology, Sanquin Research, Amsterdam, Netherlands.

Classifications MeSH