August 25, 2010British Journal of Haematology

The impact of bleeding history, von Willebrand factor and PFA–100® on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM‐1VWD

Q: What is the clinical evidence from this study?

Study design: Observational. Population: Type 1 von Willebrand disease (n=362). Intervention: PFA-100 closure time and VWF levels vs. Healthy controls and unaffected family members. Primary outcome: Relationship between PFA-100, VWF levels, and bleeding score.

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Key result

PFA-100 closure times and VWF levels were strongly related, but PFA-100 did not significantly improve the diagnostic capability of VWF antigen measurement for type 1 von Willebrand disease.

Why the study?

Does PFA-100 closure time improve the diagnosis of type 1 von Willebrand disease compared to VWF antigen measurement?

Population

362 individuals including 107 index cases of type 1 von Willebrand disease, 105 affected family members, 71…

Design

Cross-sectional

Authors

GCGiancarlo CastamanAzienda Ospedaliero-Universitaria Careggi ATAlberto TosettoVascular Medicine AGAnne GoodeveUniversity of Sheffield

Discussion

Member takes

Implication

Does not support adding PFA-100 to VWF antigen testing for type 1 VWD; leaves open its diagnostic role in other subtypes.

Study Design

Type

Observational (n=362)

Multicenter

Yes

Structured PICO

Does PFA-100 closure time improve the diagnosis of type 1 von Willebrand disease compared to VWF antigen measurement?

Population

362 participants including 107 index cases, 105 affected and 71 unaffected family members, and 79 healthy controls evaluated for type 1 von Willebrand disease.

Exposure

Measurement of Platelet Function Analyzer (PFA)-100 closure time (with epinephrine or adenosine diphosphate cartridges) and von Willebrand factor (VWF) levels

Outcome

Relationship between PFA-100 closure time, VWF levels, and bleeding score, and diagnostic capability for type 1 VWDsurrogate

PFA-100 closure time does not significantly improve the diagnostic capability of VWF antigen measurement for type 1 von Willebrand disease, though a normal result may help exclude it.

Limitations

Whether PFA-100 could replace the more specific VWF assay in patients with significant mucocutaneous bleeding symptoms remains to be investigated prospectively.

Cite This Study

Castaman et al. (2010) conducted an observational in Type 1 von Willebrand disease (n=362). PFA-100 closure time and VWF levels vs. Healthy controls and unaffected family members was evaluated on Relationship between PFA-100, VWF levels, and bleeding score. PFA-100 closure times and VWF levels were strongly related, but PFA-100 did not significantly improve the diagnostic capability of VWF antigen measurement for type 1 von Willebrand disease.

synapsesocial.com/papers/6a5e4070ffe7d48a36d89413 https://doi.org/10.1111/j.1365-2141.2010.08333.x

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The impact of bleeding history, von Willebrand factor and PFA–100® on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM‐1VWD

Discussion

Member takes

Implication

Study Design

Structured PICO

Limitations

Cite This Study

The impact of bleeding history, von Willebrand factor and PFA–100® on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM‐1VWD

Discussion

Member takes

Implication

Study Design

Structured PICO

Limitations

Cite This Study

Also Consider

Also Consider