Unresolved hemostasis issues in haemophilia.

Autor: Sidonio RF Jr; Hemophilia of Georgia Center for Bleeding and Clotting Disorders of CHOA, Atlanta, Georgia, USA., Weisel JW; University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA., Stafford D; University of North Carolina, Chapel Hill, North Carolina, USA.
Jazyk: angličtina
Zdroj: Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2024 Apr; Vol. 30 Suppl 3, pp. 70-77. Date of Electronic Publication: 2024 Apr 04.
DOI: 10.1111/hae.14999
Abstrakt: Despite rapid technological advancement in factor and nonfactor products in the prevention and treatment of bleeding in haemophilia patients, it is imperative that we acknowledge gaps in our understanding of how hemostasis is achieved. The authors will briefly review three unresolved issues in persons with haemophilia (PwH) focusing on the forgotten function that red blood cells play in hemostasis, the critical role of extravascular (outside circulation) FIX in hemostasis in the context of unmodified and extended half-life FIX products and finally on the role that skeletal muscle myosin plays in prothrombinase assembly and subsequent thrombin generation that could mitigate breakthrough muscle hematomas.
(© 2024 John Wiley & Sons Ltd.)
Databáze: MEDLINE
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