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Factor VIII : Long-established role in haemophilia A and emerging evidence beyond haemostasis

Samuelson Bannow, Bethany ; Recht, Michael ; Négrier, Claude ; Hermans, Cédric ; Berntorp, Erik LU ; Eichler, Hermann ; Mancuso, Maria Elisa ; Klamroth, Robert ; O'Hara, Jamie and Santagostino, Elena , et al. (2019) In Blood Reviews 35. p.43-50
Abstract

Factor VIII protein (FVIII) as a coagulation replacement factor has for decades been used as the standard of care for management of people with haemophilia A. It is effective for treatment of bleeding events, as prophylaxis to prevent bleeding events and preserve joint function, and to support surgery in people with haemophilia A. Despite long experience in treating haemophilia A, we are only beginning to understand the functions of FVIII beyond its established role as a coenzyme to factor IXa to expedite thrombin generation through the intrinsic pathway of coagulation. Here, we review the current role of FVIII coagulant (FVIII:C) in haemophilia A management and emerging evidence for the role of FVIII across multiple systems, including... (More)

Factor VIII protein (FVIII) as a coagulation replacement factor has for decades been used as the standard of care for management of people with haemophilia A. It is effective for treatment of bleeding events, as prophylaxis to prevent bleeding events and preserve joint function, and to support surgery in people with haemophilia A. Despite long experience in treating haemophilia A, we are only beginning to understand the functions of FVIII beyond its established role as a coenzyme to factor IXa to expedite thrombin generation through the intrinsic pathway of coagulation. Here, we review the current role of FVIII coagulant (FVIII:C) in haemophilia A management and emerging evidence for the role of FVIII across multiple systems, including the cardiovascular system, angiogenesis and maintenance of bone health. For instance, supraphysiological FVIII levels are a risk factor for venous thromboembolism. von Willebrand factor (VWF), which forms a non-covalent complex with circulating FVIII, is an established marker and regulator of angiogenesis. In a mouse model of haemophilia, treatment with FVIII decreased expression of receptor activator of nuclear factor kappa-Β ligand (RANKL), a marker for bone turnover. Longitudinal follow-up data in people with haemophilia A are needed to confirm and extend these observations.

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type
Contribution to journal
publication status
published
subject
keywords
Angiogenesis, Bone density, FVIII, Haemophilia, Prophylaxis, Thrombin generation
in
Blood Reviews
volume
35
pages
43 - 50
publisher
Churchill Livingstone
external identifiers
  • scopus:85063252296
  • pmid:30922616
ISSN
0268-960X
DOI
10.1016/j.blre.2019.03.002
language
English
LU publication?
yes
id
e4adad42-dea6-468f-ae9e-c78107b9136a
date added to LUP
2019-04-08 13:02:30
date last changed
2024-07-10 12:03:55
@article{e4adad42-dea6-468f-ae9e-c78107b9136a,
  abstract     = {{<p>Factor VIII protein (FVIII) as a coagulation replacement factor has for decades been used as the standard of care for management of people with haemophilia A. It is effective for treatment of bleeding events, as prophylaxis to prevent bleeding events and preserve joint function, and to support surgery in people with haemophilia A. Despite long experience in treating haemophilia A, we are only beginning to understand the functions of FVIII beyond its established role as a coenzyme to factor IXa to expedite thrombin generation through the intrinsic pathway of coagulation. Here, we review the current role of FVIII coagulant (FVIII:C) in haemophilia A management and emerging evidence for the role of FVIII across multiple systems, including the cardiovascular system, angiogenesis and maintenance of bone health. For instance, supraphysiological FVIII levels are a risk factor for venous thromboembolism. von Willebrand factor (VWF), which forms a non-covalent complex with circulating FVIII, is an established marker and regulator of angiogenesis. In a mouse model of haemophilia, treatment with FVIII decreased expression of receptor activator of nuclear factor kappa-Β ligand (RANKL), a marker for bone turnover. Longitudinal follow-up data in people with haemophilia A are needed to confirm and extend these observations.</p>}},
  author       = {{Samuelson Bannow, Bethany and Recht, Michael and Négrier, Claude and Hermans, Cédric and Berntorp, Erik and Eichler, Hermann and Mancuso, Maria Elisa and Klamroth, Robert and O'Hara, Jamie and Santagostino, Elena and Matsushita, Tadashi and Kessler, Craig}},
  issn         = {{0268-960X}},
  keywords     = {{Angiogenesis; Bone density; FVIII; Haemophilia; Prophylaxis; Thrombin generation}},
  language     = {{eng}},
  pages        = {{43--50}},
  publisher    = {{Churchill Livingstone}},
  series       = {{Blood Reviews}},
  title        = {{Factor VIII : Long-established role in haemophilia A and emerging evidence beyond haemostasis}},
  url          = {{http://dx.doi.org/10.1016/j.blre.2019.03.002}},
  doi          = {{10.1016/j.blre.2019.03.002}},
  volume       = {{35}},
  year         = {{2019}},
}