Unresolved hemostasis issues in haemophilia

Author:

Sidonio Robert F.1ORCID,Weisel John W.2,Stafford Darrel3ORCID

Affiliation:

1. Hemophilia of Georgia Center for Bleeding and Clotting Disorders of CHOA Atlanta Georgia USA

2. University of Pennsylvania Perelman School of Medicine Philadelphia Pennsylvania USA

3. University of North Carolina Chapel Hill North Carolina USA

Abstract

AbstractDespite 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.

Publisher

Wiley

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