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November 1, 1998Haemophilia

Optimizing therapy with factor VIII/von Willebrand factor concentrates in von Willebrand disease

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Why the study?

Are factor VIII/von Willebrand factor concentrates effective in achieving clinical haemostasis and correcting haemostatic defects in patients with von Willebrand disease?

Population

Patients with von Willebrand disease, particularly type 3 and severe cases of types 1 and 2 where…

Design

Review

Authors

FFedericiUniversity of MilanMMannucciUniversity of Milan

Discussion

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Implication

Supports FVIII/vWF concentrates for haemostasis in severe vWD; leaves open whether bleeding time normalization is required.

Key Points

  • To evaluate the pharmacokinetic properties, multimeric structure, and clinical efficacy of virus-inactivated factor VIII/von Willebrand factor concentrates in the management of von Willebrand disease.
  • Evaluated in vitro multimeric structures and pharmacokinetics of four virus-inactivated FVIII/vWF concentrates tested in a crossover randomized trial.
  • Assessed postinfusion factor VIII coagulant activity (FVIII:C), correction of prolonged bleeding time, and clinical hemostatic response during surgery and bleeding episodes.
  • No evaluated FVIII/vWF concentrate exhibited an intact multimeric structure equivalent to normal plasma or cryoprecipitate.
  • All concentrates achieved normal and sustained FVIII:C postinfusion levels, though peak levels were delayed for concentrates devoid of FVIII:C, and none achieved sustained normalization of bleeding time.
  • Effective clinical hemostasis was consistently attained during bleeding episodes and surgical procedures irrespective of bleeding time correction, with adjunctive desmopressin or platelet concentrates resolving refractory mucosal bleeding.

Structured PICO

Are factor VIII/von Willebrand factor concentrates effective in achieving clinical haemostasis and correcting haemostatic defects in patients with von Willebrand disease?

P
Population
Patients with von Willebrand disease, particularly type 3 and severe cases of types 1 and 2 where desmopressin (DDAVP) is ineffective
I
Intervention
Factor VIII/von Willebrand factor (FVIII/vWF) concentrates
O
Outcome
Correction of bleeding time (BT), factor VIII coagulant activity (FVIII:C), and clinical haemostasis

FVIII/vWF concentrates are effective for achieving clinical haemostasis in severe von Willebrand disease, even without consistent normalization of bleeding time.

Cite This Study

Federici et al. (1998) studied this question.

synapsesocial.com/papers/6a92ea7e24766d9f0a4b1094https://doi.org/10.1046/j.1365-2516.1998.0040s3007.x
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factor1985 · 185 citations
  2. 2Use of a High‐Purity Factor VIII Concentrate (Hemate P) in von Willebrand's Disease11989 · 66 citations
  3. 3Recommended Protocol for the Study of the Ex Vivo Biological Effects of Virus-Inactivated Plasma Concentrates in Patients with von Willebrand Disease1992 · 11 citations
  4. 4Comparison of four virus-inactivated plasma concentrates for treatment of severe von Willebrand disease: a cross-over randomized trial1992 · 100 citations
  5. 5DDAVP shortens the prolonged bleeding times of patients with severe von Willebrand disease treated with cryoprecipitate. Evidence for a mechanism of action independent of released von Willebrand factor1989 · 96 citations