Table 2.

GRADE test accuracy evidence summary for VWF:GPIbR in patients with suspected VWD

Sensitivity: 0.80-1.00 Specificity: 0.81-0.97  Prevalences: 3%, 20%, 50% 
Sensitivity: 0.80-1.00 Specificity: 0.81-0.97  Prevalences: 3%, 20%, 50% 
OutcomeStudies (n); patients (n)Study designFactors that may decrease certainty of evidenceEffect per 1000 patients testedTest accuracy CoE
Risk of biasIndirectnessInconsistencyImprecisionPublication biasPretest probability of 3%*Pretest probability of 20%Pretest probability of 50%§
True positives (patients with VWD) 4;404 Cohort & case-control type studies Serious Not serious Not serious Not serious None 24-30 160-200 400-500 ⨁⨁⨁◯ MODERATE 
False negatives (patients incorrectly classified as not having VWD) 0-6 0-40 0-100 
True negatives (patients without VWD) 4;575 Cohort & case-control type studies Serious Not serious Not serious Not serious None 786-941 648-776 405-485 ⨁⨁◯◯ LOW 
False positives (patients incorrectly classified as having VWD) 29-184 24-152 15-95 
 
OutcomeStudies (n); patients (n)Study designFactors that may decrease certainty of evidenceEffect per 1000 patients testedTest accuracy CoE
Risk of biasIndirectnessInconsistencyImprecisionPublication biasPretest probability of 3%*Pretest probability of 20%Pretest probability of 50%§
True positives (patients with VWD) 4;404 Cohort & case-control type studies Serious Not serious Not serious Not serious None 24-30 160-200 400-500 ⨁⨁⨁◯ MODERATE 
False negatives (patients incorrectly classified as not having VWD) 0-6 0-40 0-100 
True negatives (patients without VWD) 4;575 Cohort & case-control type studies Serious Not serious Not serious Not serious None 786-941 648-776 405-485 ⨁⨁◯◯ LOW 
False positives (patients incorrectly classified as having VWD) 29-184 24-152 15-95 
 
*

Typically seen in patients investigated for VWD because of a personal history of bleeding symptoms (eg, mucocutaneous bleeding) (Quiroga, 2007).46 

Typically seen in patients investigated for VWD as a first-degree relative of a patient with VWD.

Serious patient selection risk of bias due to case-control design. Also, 3 of the 4 studies (Boender [2018],47  Vangenechten [2018],48  and Sagheer [2016])49  investigated test accuracy for classifying type 2 VWD patients (using a ratio of 0.6), not for diagnosing VWD.

§

Typically seen in patients investigated for VWD because of a personal history of abnormal laboratory test (eg, increased partial thromboplastin time).

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