Assessment of primary haemostasis with a new recombinant von Willebrand factor in patients with von Willebrand disease

Trossaërt, Marc; Flaujac, Claire; Jeanpierre, Emmanuelle; Drillaud, Nicolas; Sigaud, Marianne; Fouassier, Marc; Ternisien, Catherine; Raucourt, Emmanuelle de

doi:10.1111/hae.13916

Cited by 5 publications

(8 citation statements)

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“…One fourth of these women experienced blood loss meeting criteria for PPH per 2017 US American College of Obstetrics and Gynecology guidelines, a rate observed in only 3% to 5% of non-VWD obstetric patients. 18 These data confirm that despite pregnancy-associated increases in VWF levels, and despite higher VWF dosing of 80 IU/kg, with rVWF or pdVWF at delivery, postpartum bleeding occurs. Furthermore, there is no apparent relationship between VWF level and PPH risk.…”

Section: Discussionsupporting

confidence: 73%

“…A recent case series of 2 patients with severe VWD, 1 with type 3 VWD requiring prophylaxis for severe mucosal bleeding and the other with type 2A undergoing knee replacement surgery, reported higher FVIII and VWF levels with longer half-life compared with historical treatment of patients with pdVWF and, in the latter patient, higher and longer-lasting high-molecular weight multimers vs those with pdVWF for similar surgery 3 years previously. 18 Whether rVWF reduces postpartum bleeding to a greater degree than pdVWF has not been studied. We, therefore, conducted a retrospective observational study of delivery outcomes in women with VWD at a single institution, comparing rVWF and pdVWF.…”

Section: Introductionmentioning

confidence: 99%

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Recombinant vs plasma-derived von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease

Machin

Ragni

2020

Blood Advances

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von Willebrand disease (VWD) is a congenital bleeding disorder characterized by deficient or defective von Willebrand factor (VWF). Among women with VWD, postpartum hemorrhage (PPH) is common. Treatment options at delivery include plasma-derived VWF (pdVWF) and recombinant VWF (rVWF). However, limited data are available regarding their efficacy. We conducted a retrospective observational study comparing PPH in women with VWD treated at the Hemophilia Center of Western Pennsylvania between 1 February 2017 and 31 January 2018 with either rVWF or pdVWF. We compared postpartum outcomes, including PPH frequency and estimated blood loss (EBL) at delivery. There were a total of 12 deliveries, 7 vaginal and 5 cesarean. At delivery and for 3 days postpartum, 6 women received 80 IU/kg of rVWF and 6 received 80 IU/kg of pdVWF, based on prepregnancy weight, insurance, and/or patient choice. Treatment groups had similar demographics, including median age (32.0 vs 27.0 years; P = .075), bleeding scores (3.0 vs 3.5; P = .734), and prepregnancy body mass index (29.0 vs 29.2 kg/m2; P = .691). PPH occurred in 3 (25.0%) of 12 deliveries, with no difference by treatment group (2 of 6 rVWF vs 1 of 6 pdVWF; P = 1.000) and no difference in EBL by treatment group (685 vs 462 mL; P = .384) or delivery type (vaginal, P = .722 vs cesarean, P = .531). In summary, PPH occurred in one-fourth of the deliveries in women with VWD, despite a higher dose (80 IU/kg) of rVWF or pdVWF. Future trials are needed to develop and assess novel strategies to prevent PPH in VWD.

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Section: Discussionsupporting

confidence: 73%

Section: Introductionmentioning

confidence: 99%

Recombinant vs plasma-derived von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease

Machin

Ragni

2020

Blood Advances

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“…Previous reports showed that the proportion of ULM increased from 0 to 30% 15 minutes after the infusion of rVWF, then declined substantially between 12 and 24 hours, possibly providing an improvement in primary haemostasis. 7,10 Some authors recommend to monitor only FVIII:C although others recommend to monitor VWF activity. 19,20 As it was a ret- In this study, it seems clear that there is a great deal of heterogeneity in the characteristics of VWD patients and the use of rVWF by different physicians.…”

Section: Discussionmentioning

confidence: 99%

“…The demographic characteristics of the patients are detailed in Table 1. For the 63 surgeries, the median rVWF dosages used were 63 (12-340) IU/kg and the median number of exposure days (ED) was 2 (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”

Section: Patientsmentioning

confidence: 99%

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Efficacy and safety of a recombinant Von Willebrand Factor treatment in patients with inherited Von Willebrand Disease requiring surgical procedures

et al. 2021

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Introduction: Von Willebrand Disease is a common inherited haemorrhagic disorder due to a deficiency of Von Willebrand Factor (VWF). In case of surgical procedures in patients who are not responsive or have contraindications to desmopressin, replacement therapy with VWF concentrates is indicated. Until recently, only plasma-derived VWF concentrates were available. A new recombinant VWF (rVWF) concentrate that contains no Factor VIII (FVIII) but a high amount of high molecular weight VWF multimers has been available in France since 2018. Aim: Describe real-world experience of using rVWF in surgical procedures. Methods: Sixty-three surgeries for 55 patients were retrospectively analysed in 7 French haemostasis centres. Results: During minor surgeries, the median (range) number of infusions was 1 (1-8) with a preoperative loading dose of 35 (19-56) rVWF IU/kg and a total median dose of 37.5 IU (12-288). During major surgeries, the median (range) number of infusions was only 3 (1-14) with a median preoperative loading dose of 36 IU (12-51) rVWF IU/kg, and a total median dose of 108 IU (22-340) rVWF IU/kg. The overall clinical efficacy was qualified as excellent/good in 61 of the procedures (97%), moderate in 1 (1.5%) and poor in 1 (1.5%). There was no accumulation of VWF or FVIII during postoperative monitoring. No thromboembolic events, anti-VWF antibodies or adverse events were reported. Conclusion: This French 'real-world' experience shows that a few infusions and low doses of rVWF provided effective prevention of bleeding in major and minor surgeries in inherited VWD, with no clinically significant safety concerns. K E Y W O R D S inherited von Willebrand disease, recombinant VWF, surgical procedure | 271 DESPREZ Et al. How to cite this article: Desprez D, Drillaud N, Flaujac C, et al. Efficacy and safety of a recombinant Von Willebrand Factor treatment in patients with inherited Von Willebrand Disease requiring surgical procedures. Haemophilia.

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Point-of-Care Testing in Patients with Hereditary Disorders of Primary Hemostasis: A Narrative Review

Bavinck,

Heerde,

Schols

2024

Semin Thromb Hemost

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Inherited disorders of primary hemostasis, such as von Willebrand disease and congenital platelet disorders, can cause extensive, typically mucocutaneous bleeding. Assays to diagnose and monitor these disorders, such as von Willebrand factor activity assays and light transmission aggregometry, are performed in specialized hemostasis laboratories but are commonly not available in local hospitals. Due to the complexity and relative scarcity of these conventional assays, point-of-care tests (POCT) might be an attractive alternative in patients with hereditary bleeding disorders. POCTs, such as thromboelastography, are increasingly used to assess hemostasis in patients with acquired hemostatic defects, aiding clinical decision-making in critical situations, such as during surgery or childbirth. In comparison, the use of these assays in patients with hereditary hemostasis defects remains relatively unexplored. This review aims to give an overview of point-of-care hemostasis tests in patients with hereditary disorders of primary hemostasis. A summary of the literature reporting on the performance of currently available and experimental POCTs in these disorders is given, and the potential utility of the assays in various use scenarios is discussed. Altogether, the studies included in this review reveal that several POCTs are capable of identifying and monitoring severe defects in the primary hemostasis, while a POCT that can reliably detect milder defects of primary hemostasis is currently lacking. A better understanding of the strengths and limitations of POCTs in assessing hereditary defects of primary hemostasis is needed, after which these tests may become available for clinical practice, potentially targeting a large group of patients with milder defects of primary hemostasis.

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Assessment of primary haemostasis with a new recombinant von Willebrand factor in patients with von Willebrand disease

Cited by 5 publications

References 10 publications

Recombinant vs plasma-derived von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease

Recombinant vs plasma-derived von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease

Efficacy and safety of a recombinant Von Willebrand Factor treatment in patients with inherited Von Willebrand Disease requiring surgical procedures

Point-of-Care Testing in Patients with Hereditary Disorders of Primary Hemostasis: A Narrative Review

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