Pharmacokinetics of von Willebrand factor and factor VIII coagulant activity in patients with von Willebrand disease type 3 and type 2

Ménaché, D

doi:10.1046/j.1365-2516.1998.0040s3044.x

Cited by 17 publications

(22 citation statements)

References 13 publications

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“…The IVR was consistent over doses ranging from 15 to 151 IU kg −1 . The median terminal half‐life of VWF:RCo determined in this study was 15.6 h, in line with values reported in several smaller previous studies of 11.3 [16], 11.3–13.9 [33] and 12 h [26].…”

Section: Discussionsupporting

confidence: 92%

von Willebrand factor/factor VIII concentrate (Haemate® P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery

Lethagen

Kyrle

Castaman

et al. 2007

Journal of Thrombosis and Haemostasis

111

165

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factor/factor VIII concentrate (Haemate R P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery. J Thromb Haemost 2007; 5: 1420-30.Summary. Background: While plasma-derived concentrates containing large amounts of von Willebrand factor (VWF) are effective in treating von Willebrand disease (VWD), optimal dosing remains to be fully characterized. Objectives: To determine the feasibility of dosing Haemate P Ò VWF/factor VIII (FVIII) concentrate based on pharmacokinetics (PK) in the management of surgical subjects with VWD. Methods: VWD subjects scheduled for elective surgery were enrolled in a prospective multicenter open-label cohort study. A pre-operative loading dose of VWF/FVIII concentrate based upon prior individual subject PK analysis was administered followed by postoperative therapeutic/maintenance infusions. Results: Twenty-eight subjects with types 1, 2A or 3 VWD and one with type 2 M were enrolled. Median in vivo recovery of VWF ristocetin cofactor (VWF:RCo) was 1.9 IU dL )1 (IU kgwith an interquartile range (IQR) of 1.6-2.5 IU dL. Median response, half-life and clearance were 74.0% (IQR, 55.5-100%), 15.6 h (IQR, 9.0-28.4 h) and 3.26 mL kg ) was administered. Postoperative mean trough VWF:RCo levels of 62-73 IU dL )1 were sufficient to prevent bleeding. Investigators rated hemostasis excellent or good in 96.3% of subjects on the day of surgery and 100% on the next day and on day 14. A subject with multiple risk factors developed pulmonary embolism, which resolved without sequelae. Conclusions: Haemate P Ò provided effective and safe hemostasis in VWD subjects undergoing elective surgery. Selection of Haemate Ò P loading dose on the basis of VWF PK proved feasible.

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Section: Discussionsupporting

confidence: 92%

von Willebrand factor/factor VIII concentrate (Haemate® P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery

Lethagen

Kyrle

Castaman

et al. 2007

Journal of Thrombosis and Haemostasis

111

165

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“…In another study , nine patients with VWD type 3, six with type 2B, one with type 2A, and one patient with type 1/2N were infused with one dose of approximately 50 or 100 IU ristocetin cofactor activity (RCoF) per kg of VWF (Human), a product with a very low content of FVIII. The FVIII:C rate of synthesis was found to be 6.4 U dL −1 h −1 (range: 4.4–8.8).…”

Section: A Lifespan Of Pharmacokinetics In Vwdmentioning

confidence: 99%

Third Åland islands conference on vonWillebrand disease, 26–28September 2012: meeting report

Berntorp

Fuchs²,

Makris

et al. 2013

Haemophilia

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Summary The first meeting of international specialists in the field of von Willebrand disease (VWD) was held in the Åland islands in 1998 where Erik von Willebrand had first observed a bleeding disorder in some members of a family from Föglö and a summary of the meeting was published in 1999. The second meeting was held in 2010 and a report of the meeting was published in 2012. Topics covered included progress in understanding of VWD over the last 50 years; multimers; classification of VWD; pharmacokinetics and laboratory assays; genetics; treating the paediatric patient; prophylaxis; geriatrics; gene therapy and treatment guidelines. This third meeting held over 3 days covered the structure and function of von Willebrand factor (VWF); type 1 VWD, the most common form of the disease; a lifespan of pharmacokinetics in VWD; detecting inhibitors in VWD patients; and special challenges in understanding and treating the female VWD patient.

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“…Based on studies in severe (type 3) vWD patients, these infusions result in a very long apparent factor VIII half-life, approximately 40 hours, compared with approximately 12 hours for vWF antigen and activity. 39 The FDA now requires that plasma products approved for use in vWD be labeled in terms of vWF, and hematologists are starting to become familiar with prescribing and monitoring therapy in ristocetin cofactor (RCof) units. The recommended dosage is 40-80 RCof units/kg depending on the severity of bleeding.…”

Section: Acquired Hemophiliamentioning

confidence: 99%

Unexpected Bleeding Disorders

Aledort

Teitel

2001

Hematology

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Patients with known coagulation deficiencies, either congenital or acquired, may bleed spontaneously with trauma or with surgical intervention. In contrast, however, are the unchallenged patients who bleed in a variety of clinical settings that demand rapid diagnosis so that appropriate therapy can be instituted.In the first section Dr. Louis M. Aledort demonstrates a series of vignettes of actual cases who presented with unexpected bleeding or a screening laboratory abnormality prior to a needed surgical intervention. Settings include dental, oral surgical, obstetrical, surgical and gynecological. The differential diagnoses of these cases are discussed.In the second section Dr. David Green also uses vignettes to demonstrate how the laboratory is used to differentiate the various clinical entities. The choice and priority of required tests indicated by the settings, history, site and type of bleeding, and the syllogisms used to define the abnormality are stressed.In the third section, Dr. Jerome Teitel reviews in detail the therapeutic armamentarium available to the clinician and presents algorithms for the management of these bleeding disorders.

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Pharmacokinetics of von Willebrand factor and factor VIII coagulant activity in patients with von Willebrand disease type 3 and type 2

Cited by 17 publications

References 13 publications

von Willebrand factor/factor VIII concentrate (Haemate® P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery

von Willebrand factor/factor VIII concentrate (Haemate® P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery

Third Åland islands conference on vonWillebrand disease, 26–28September 2012: meeting report

Unexpected Bleeding Disorders

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