Determination of High-Molecular-Weight von Willebrand factor multimers and their impact on specific VWF-activities in VWF/FVIII-concentrates

Walter, Olga Maria Formigoni Carvalho; Budde, Ulrich; Muysers, C.; Metzner, Hubert J.; Suiter, Tobias M.

doi:10.1111/j.1538-7836.2003.tb05768.x

Cited by 8 publications

(10 citation statements)

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“…2b) was characterized by predominant central bands and satellite bands of similar intensity, indicating that the VWF molecules were not proteolysed during the additional steps included in the process. This multimeric pattern is similar to that of normal plasma and contrasts with that recently observed in other commercial FVIII/VWF concentrates showing signs of increased proteolysis [14,15, O. Magurier, unpublished].…”

Section: Resultssupporting

confidence: 77%

In vitro study of a triple‐secured von Willebrand factor concentrate

et al. 2004

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Section: Resultssupporting

confidence: 77%

In vitro study of a triple‐secured von Willebrand factor concentrate

et al. 2004

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“…The HMW multimers of VWF are thought to be critical in correcting the coagulation defects in patients with VWD (115, 137, 140, 148, 151–154). Multimer analyses have revealed that Haemate ® P contains relatively high levels of intermediate and HMW multimers of VWF, unlike most other VWF/FVIII products in which predominantly the lower and some intermediate‐molecular‐weight multimers are found (112–116, 155).…”

Section: Overview Of Haemate®pmentioning

confidence: 99%

A systematic overview of the first pasteurised VWF/FVIII medicinal product, Haemate^®P/ Humate^®‐P: History and clinical performance

Berntorp

Archey

Auerswald

et al. 2008

European J of Haematology

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Patients with von Willebrand disease (VWD) and haemophilia A (HA) lack, to varying degrees, the von Willebrand factor (VWF) and coagulation factor VIII (FVIII) that are critical for normal haemostasis. These conditions in turn make patients prone to uncontrolled bleeding. Historically, patients with severe forms of VWD or HA were crippled before adulthood and their life expectancy was significantly reduced. Over the past decades, specific coagulation factor replacement therapies including Haemate Ò P, have been developed to help patients achieve and maintain normal haemostasis. Haemate Ò P is a human, plasma-derived VWF ⁄ FVIII medicinal product, which was first licensed in Germany in 1981 for the treatment of HA-associated bleeding. It has since then come to be accepted as the gold standard for both the treatment and prophylaxis of bleeding in VWD, especially in cases where desmopressin [1-deamino-8-D-arginine vasopressin (DDAVP)] has been ineffective. Haemate Ò P was the first effectively virus-inactivated (pasteurisation: 60°C for 10 h in aqueous solution) FVIII product, whereby the risk of potentially threatening infective complications of plasma-derived products was reduced. Haemate Ò P was also shown to have a VWF multimer profile remarkably close to that of normal plasma. This bibliographic review presents previously unpublished clinical data of Haemate Ò P, based upon internal clinical study reports of the proprietor, CSL Behring, in addition to data already presented in other publications. The data demonstrate a predictable and well-characterised pharmacokinetic profile, and a proven record of short-and long-term safety, while effectively correcting the haemostatic defects in VWD and HA. Recently available data have also shown Haemate Ò P to be of haemostatic value in exceptional clinical circumstances including surgical interventions. By virtue of its plasma-derived combination of VWF and FVIII, in addition to its high VWF:FVIII content ratio (2.4:1), Haemate Ò P is also associated with successful immune tolerance induction in those patients developing inhibitor antibodies. Although the theoretical risk of thromboembolic complications does exist while receiving Haemate Ò P, as it does with any FVIII replacement therapy, the incidence of such complications has remained notably low. Given the robust data that have accumulated for the use of Haemate Ò P, dosing recommendations are also described in this review; the recommendations are tailored to patient-specific contexts including baseline VWF and FVIII levels in plasma and the type of surgical intervention being undertaken. A wide variety of studies have also provided data on paediatric and geriatric populations, all of which have suggested that Haemate Ò P can be safely and effectively used in a wide variety of clinical circumstances. Effective haemostasis requires complex bodily processes such as vasoconstriction and blood clotting so as to ensure the integrity of the blood vessel system and to prevent excessive blood loss following injury. B...

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“…A comparative gel electrophoresis study of 12 VWF/FVIII concentrates demonstrated that the HMW VWF multimer pattern in Haemate P/Humate‐P (94%) was similar to that in normal human plasma (100%), whereas other concentrates showed a deficit in this multimer fraction (e.g. 79% in Innobrand, and from 4% to 36% in the other 10 products examined) [9,10]. High molecular‐weight multimers of VWF play an important role in early haemostasis and thrombus formation, particularly in vascular lesions under high shear stress conditions [11].…”

Section: Clinical Pharmacologymentioning

confidence: 99%

Haemate^® P/Humate‐P^® for the treatment of von Willebrand disease: considerations for use and clinical experience

Auerswald

Kreuz

2008

Haemophilia

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von Willebrand disease (VWD) is a heterogeneous bleeding disorder with symptoms in affected patients ranging from mild effects to potentially devastating haemorrhagic events. Desmopressin (DDAVP) and von Willebrand factor/factor VIII (VWF/FVIII) concentrates are the principal treatments. Haemate P/Humate-P is an intermediate-purity VWF/FVIII concentrate with extensive clinical experience in VWD. This concentrate has been shown to correct haemostatic defects of VWD, with efficacy ratings of good/excellent in nearly all patients treated for bleeding or surgical events. Haemate P/Humate-P has a high content of the high molecular weight (HMW) VWF multimer fraction, which has been shown to be very effective in achieving haemostasis. The HMW VWF multimer pattern in Haemate P/Humate-P is more similar to that of normal human plasma (94% for Haemate P/Humate-P vs. 100% for normal human plasma) than that of other VWF/FVIII concentrates and correlates with functional VWF activities including ristocetin cofactor activity (VWF:RCo) and collagen-binding activity. The recommended dosing of Haemate P/Humate-P is based preferentially on VWF:RCo activity, which is approximately twice that of FVIII:C (2.4:1). Haemate P/Humate-P has been shown to be safe; no serious adverse events or cases of thrombosis have been observed in clinical trials and no documented cases of viral transmission in nearly three decades of clinical use. While DDAVP is effective in a large proportion of VWD patients, it may not provide adequate haemostasis in all situations. In such cases, Haemate P/Humate-P is an effective replacement concentrate for all types of VWD in both adult and paediatric patients.

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Determination of High-Molecular-Weight von Willebrand factor multimers and their impact on specific VWF-activities in VWF/FVIII-concentrates

Cited by 8 publications

References 0 publications

In vitro study of a triple‐secured von Willebrand factor concentrate

In vitro study of a triple‐secured von Willebrand factor concentrate

A systematic overview of the first pasteurised VWF/FVIII medicinal product, Haemate^®P/ Humate^®‐P: History and clinical performance

Haemate^® P/Humate‐P^® for the treatment of von Willebrand disease: considerations for use and clinical experience

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Determination of High-Molecular-Weight von Willebrand factor multimers and their impact on specific VWF-activities in VWF/FVIII-concentrates

Cited by 8 publications

References 0 publications

In vitro study of a triple‐secured von Willebrand factor concentrate

In vitro study of a triple‐secured von Willebrand factor concentrate

A systematic overview of the first pasteurised VWF/FVIII medicinal product, Haemate®P/ Humate®‐P: History and clinical performance

Haemate® P/Humate‐P® for the treatment of von Willebrand disease: considerations for use and clinical experience

Contact Info

Product

Resources

About

A systematic overview of the first pasteurised VWF/FVIII medicinal product, Haemate^®P/ Humate^®‐P: History and clinical performance

Haemate^® P/Humate‐P^® for the treatment of von Willebrand disease: considerations for use and clinical experience