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Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease
M Bowman1, G Mundell, J Grabell
1Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada.
Insights
A condensed bleeding questionnaire efficiently evaluates patients for von Willebrand disease (VWD). This validated tool accurately quantifies hemorrhagic symptoms, improving diagnostic efficiency for VWD.
Area of Science:
- Hematology
- Medical Diagnostics
- Clinical Research
Background:
- Accurate bleeding histories are challenging to obtain, necessitating standardized methods for quantifying hemorrhagic symptoms.
- The value of standardized bleeding assessment in diagnosing bleeding disorders has been recognized.
Purpose of the Study:
- To condense an extensive validated bleeding questionnaire (MCMDM-1VWD) while retaining diagnostic accuracy for bleeding score (BS).
- To evaluate the diagnostic utility and reliability of the condensed bleeding questionnaire in patients evaluated for von Willebrand disease (VWD).
Main Methods:
- An extensive validated bleeding questionnaire (MCMDM-1VWD) was condensed by retaining only items directly impacting the bleeding score (BS).
- The correlation between the full and condensed questionnaire versions was assessed.
- The diagnostic utility of the condensed version was prospectively tested in 259 individuals, including 217 evaluated for VWD.
Main Results:
- The condensed questionnaire demonstrated excellent correlation with the full version (Spearman's 0.97).
- Prospective testing showed a sensitivity of 100% and specificity of 87% for identifying VWD, with a negative predictive value of 1.
- The condensed version exhibited reasonable inter-observer reliability (Spearman's 0.72) and significant differences in BS across VWD subtypes, inversely correlating with VWF:Ag levels.
Conclusions:
- The Condensed MCMDM-1VWD Bleeding Questionnaire is an efficient and effective tool for evaluating patients suspected of having VWD.
- This condensed version maintains high diagnostic accuracy and reliability, simplifying the assessment of hemorrhagic symptoms.
Background:
Given the challenges involved in obtaining accurate bleeding histories, attempts at standardization have occurred and the value of quantifying hemorrhagic symptoms has been recognized.
Patients/Methods:
An extensive validated bleeding questionnaire (MCMDM-1VWD) was condensed by eliminating all details that did not directly affect the bleeding score (BS) and the correlation between the two versions was tested. Additionally, the diagnostic utility of the condensed version was prospectively tested.
Results:
Data on 259 individuals who were administered the questionnaire are presented here; 217 being prospectively investigated for von Willebrand disease (VWD) (group 1) and 42 previously known to have type 1, 2 or 3 VWD (group 2). Of the 217 prospectively investigated, 35 had positive BS (> or =4) and 182 had negative scores. Seven individuals (all with positive BS) had laboratory results consistent with type 1 VWD. This results in a sensitivity of 100% and a specificity of 87%. The positive predictive value is 0.20 and the negative predictive value is 1. The correlation between the full MCMDM-1VWD and condensed versions is excellent (Spearman's 0.97, P < 0.001, linear regression r(2) = 96.4). Inter-observer reliability for the condensed version is reasonable (Spearman's 0.72, P < 0.001 and intra-class correlation coefficient 0.805, P < 0.001). There was a significant difference in BS between subtypes of VWD, with type 3 >> type 2 >> type 1 VWD (anova P < 0.001). There is a strong inverse relationship between VWF:Ag level and BS (Spearman's -0.411, P < 0.001).
Conclusions:
The Condensed MCMDM-1VWD Bleeding Questionnaire is an efficient, effective tool in the evaluation of patients for VWD.
