A new hemophilia carrier nomenclature to define hemophilia in women and girls: communication from the SSC of the ISTH

KPM van Galen, R d'Oiron, P James… - … of Thrombosis and …, 2021 - Wiley Online Library
Hemophilia A and B predominantly attracts clinical attention in males due to X‐linked
inheritance, introducing a bias toward female carriers to be asymptomatic. This common …

[HTML][HTML] Hemophilia a

BA Konkle - 2017 - europepmc.org
Hemophilia A is characterized by deficiency in factor VIII clotting activity that results in
prolonged oozing after injuries, tooth extractions, or surgery, and delayed or recurrent …

Women and bleeding disorders: diagnostic challenges

PD James - Hematology 2014, the American Society of …, 2020 - ashpublications.org
Women with bleeding disorders suffer from multiple bleeding symptoms, including easy
bruising, epistaxis, bleeding from minor wounds and the oral cavity, and bleeding after …

Women and girls with haemophilia: lessons learned

R d'Oiron, S O'Brien, AH James - Haemophilia, 2021 - Wiley Online Library
Severe and moderate factor VIII (FVIII) or IX (FIX) deficiencies in female carriers of
haemophilia are rarely observed, but mild deficiency is quite frequent, although insufficiently …

Big picture initiatives in bleeding disorders

A Rauch, LA Valentino, K Mills, ML Witkop… - …, 2022 - Wiley Online Library
Introduction The inherited bleeding disorders (IBD) community has witnessed significant
therapeutic advances recently, yet important gaps persist, particularly for those with rare …

Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia?

S Raso, C Lambert, A Boban, M Napolitano… - …, 2020 - Wiley Online Library
Introduction Certain haemophilia carriers demonstrate an increased bleeding tendency,
mainly related to clotting factor deficiency. No study has so far formally compared the …

The von Willebrand factor–binding aptamer rondaptivon pegol as a treatment for severe and nonsevere hemophilia A

C Ay, KD Kovacevic, D Kraemmer, C Schoergenhofer… - Blood, 2023 - ashpublications.org
Factor VIII (FVIII) circulates in a noncovalent complex with von Willebrand Factor (VWF), the
latter determining FVIII half-life. The VWF-binding aptamer rondaptivon pegol (BT200) …

Regulation and importance of factor VIII levels in hemophilia A carriers

PH Cygan, PA Kouides - Current Opinion in Hematology, 2021 - journals.lww.com
Prevalence and bleeding tendency of hemophilia A carriers remain underreported, despite a
significant proportion having low FVIII levels. Skewed XCI may explain low FVIII but does not …

Enhanced VWF clearance in low VWF pathogenesis: limitations of the VWFpp/VWF: Ag ratio and clinical significance

D Doherty, M Lavin, M Byrne, M Nolan… - Blood …, 2023 - ashpublications.org
Increased von Willebrand factor (VWF) clearance plays a key role in the pathogenesis of
type 1 and type 2 von Willebrand disease (VWD). However, the pathological mechanisms …

The role of viscoelastic hemostatic assays for postpartum hemorrhage management and bedside intrapartum care

D Katz, M Farber, C Getrajdman, J Hamburger… - American Journal of …, 2023 - Elsevier
Viscoelastic hemostatic assays are point-of-care devices that assess coagulation and
fibrinolysis in whole blood samples. These technologies provide numeric and visual …