Continuous infusion therapy with very high purity von Willebrand factor concentrate in patients with severe von Willebrand disease

Mp, Smith; Km, Rice; Es, Bromidge; M, Lawn; R, Beresford-Webb; Spence, K J; Khair, Kate; Hann, Ian; Gf, Savidge

doi:10.1097/00001721-199701000-00002

Cited by 21 publications

(15 citation statements)

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“…Whereas VWF:Ag remained stable over the 24-h observation period, VWF:Rco values decreased slightly after 12 h in that study [4], which has also been observed in our patient after the dose of Haemate P was reduced at day 6. VWF:Rco remained very low between days 9 and 13, while VWF:Ag was higher.…”

Section: Discussionsupporting

confidence: 88%

“…High-purity VWF concentrates are known to be stable at 24 h after reconstitution with no evidence of neoantigen expression, as detailed with serial VWF:Rco and VWF:Ag measurements and with serial multimer pro® les [4]. Whereas VWF:Ag remained stable over the 24-h observation period, VWF:Rco values decreased slightly after 12 h in that study [4], which has also been observed in our patient after the dose of Haemate P was reduced at day 6.…”

Section: Discussionmentioning

confidence: 99%

“…VWF:Rco remained very low between days 9 and 13, while VWF:Ag was higher. In addition to the mentioned study [4] investigated the sterility of factor concentrates reconstituted and stored at room temperature for up to 24 h. The results showed no growth of cultures. Prospective clinical trials are needed to investigate the management of patients with VWD after surgery or major bleeding episodes by continuous infusion of factor concentrates.…”

Section: Discussionmentioning

confidence: 99%

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Continuous Infusion of Von Willebrand Factor and Factor Viii After Elective Heart Surgery in a 12-Year-Old Girl With Von Willebrand Disease Type 3

Kühne

Imbach²,

Marbet

et al. 1999

Pediatric Hematology and Oncology

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The continuous infusion of von Willebrand factor (VWF) in a 12-year-old girl with type 3 von Willebrand disease is described. The patient had elective heart surgery with cardiopulmonary bypass for closure of her atrial septum defect. The surgical procedure lasted 3 h. A presurgical bolus followed by a postoperative continuous infusion of factor VIII/VWF concentrates was administered. During the continuous infusion of clotting factors, stable plasma levels of hemostasis and avoidance of dangerously low levels of factor concentrates were achieved. No peri- or postsurgical complications occurred. Continuous infusion of clotting factors allows constant and hemostatic factor concentrations to be maintained with the possibility of dose titration and adjustment.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Continuous Infusion of Von Willebrand Factor and Factor Viii After Elective Heart Surgery in a 12-Year-Old Girl With Von Willebrand Disease Type 3

Kühne

Imbach²,

Marbet

et al. 1999

Pediatric Hematology and Oncology

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“…An alternative approach is to use a continuous infusion of either a VWF/FVIII containing concentrate or a VHP‐VWF concentrate. This strategy results in a good correction of the haemostatic defect of VWD and has been shown to be clinically effective in type 3 VWD [40,46]. It may also lead to a decrease in the amount of FVIII/VWF used [40].…”

Section: Type 3 Vwdmentioning

confidence: 99%

Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization

et al. 2004

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Summary. von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to inform individuals making choices about the treatment and management of VWD including the use of therapeutic products. This is the second edition of this UK Haemophilia Centre Doctors' Organization (UKHCDO) guideline and supersedes the previous edition which was published in 1994.

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“…The LFB‐von Willebrand factor (vWF) concentrate is the only available plasma‐derived product especially designed and intended for the treatment of von Willebrand disease. Since 1989, more than 40 million units have been manufactured in the LFB facility of Lille (France) and have been infused to stop or prevent bleeding in various types of von Willebrand disease patients [ 1–3]. The purification procedure is mainly based on a ion‐exchange chromatography [ 4, 5] which allows one to obtain, from the solvent/detergent (S/D) – treated cryoprecipitate, two different fractions containing vWF and procoagulant factor VIII (FVIII), which are subsequently manufactured as therapeutic products indicated for von Willebrand disease and haemophilia A, respectively.…”

Section: Introductionmentioning

confidence: 99%

In vitro evaluation of the haemostatic value of the LFB‐von Willebrand factor concentrate

Mazurier

1998

Haemophilia

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The structural and functional studies of the concentrate of von Willebrand factor (vWF) manufactured by LFB have been first performed as part of the preclinical development of this product specially intended for the treatment of von Willebrand disease. The electrophoretic analyses showed the high purity of LFB-vWF concentrate (specific activity of 100 IU of ristocetin cofactor activity per milligram of protein) and the multimeric pattern attesting the presence of high molecular weight multimeters (> or = 15-mers). The measurements of vWF capacity to bind to: (1) platelet glycoprotein (GP) Ib in the presence of ristocetin; (2) GPIIb/IIIa in the presence of thrombin; (3) types I and III collagen; (4) factor VIII (FVIII) gave evidence that the functional activity of purified vWF was similar to that of native plasma vWF. Furthermore the LFB-vWF concentrate was able to promote platelet adhesion to collagen. The release of the therapeutic batches of this product rely presently on their potency measured with the ristocetin cofactor activity (40-70 IU ml-1) and the quantitative evaluation of vWF multimeric distribution studied using electrophoresis in 1.5% agarose. These validated techniques ensure the consistency and haemostatic properties of the different lots of LFB-vWF concentrate.

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Continuous infusion therapy with very high purity von Willebrand factor concentrate in patients with severe von Willebrand disease

Cited by 21 publications

References 0 publications

Continuous Infusion of Von Willebrand Factor and Factor Viii After Elective Heart Surgery in a 12-Year-Old Girl With Von Willebrand Disease Type 3

Continuous Infusion of Von Willebrand Factor and Factor Viii After Elective Heart Surgery in a 12-Year-Old Girl With Von Willebrand Disease Type 3

Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization

In vitro evaluation of the haemostatic value of the LFB‐von Willebrand factor concentrate

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