Predictors of von Willebrand disease diagnosis in individuals with borderline von Willebrand factor plasma levels
Articolo
Data di Pubblicazione:
2015
Citazione:
Predictors of von Willebrand disease diagnosis in individuals with borderline von Willebrand factor plasma levels / P. Bucciarelli, S.M. Siboni, F. Stufano, E. Biguzzi, M.T. Canciani, L. Baronciani, M.T. Pagliari, S. La Marca, C. Mistretta, F.R. Rosendaal, F. Peyvandi. - In: JOURNAL OF THROMBOSIS AND HAEMOSTASIS. - ISSN 1538-7933. - 13:2(2015 Feb 26), pp. 228-238. [10.1111/jth.12799]
Abstract:
BACKGROUND: In individuals with borderline von Willebrand factor (VWF) plasma levels, second-level tests are required to confirm or exclude von Willebrand disease (VWD). These tests are time-consuming and expensive.
OBJECTIVE: To assess which parameters can predict VWD diagnosis in individuals with borderline VWF levels (30-60IUdL(-1) ).
METHODS: Nine hundred and fifty individuals with bleeding episodes or abnormal coagulation test results were investigated with first-level tests (blood count, prothrombin time, activated partial thromboplastin time, blood clotting factorVIII, VWF ristocetin cofactor activity [VWF:RCo], and VWF antigen), and 93 (62 females and 31 males; median age, 28years; interquartile range15-44) had borderline VWF:RCo levels. All underwent second-level investigations to confirm or exclude VWD. A multivariable logistic regression model was fitted with sex, age, bleeding score, family history, VWF:RCo and ABO blood group as predictors, and used to predict VWD diagnosis.
RESULTS: Forty-five of the 93 individuals (48%) had VWD (84% type1). A negative linear relationship between VWF:RCo levels and risk of VWD diagnosis was present, and was particularly evident with blood group non-O [adjusted odds ratio7.00 (95% confidence interval [CI]1.48-33.11) for every 5IUdL(-1) decrease in VWF:RCo]. The other variable clearly associated with VWD diagnosis was female sex (adjusted odds ratio5.76 [95%CI1.47-22.53]). The area under the receiver operating characteristic curve of the full logistic model was 0.89 (95%CI0.82-0.95).
CONCLUSIONS: In individuals with borderline VWF, the two strongest predictors of VWD diagnosis are low VWF:RCo levels (particularly in those with blood group non-O) and female sex. This predictive model has a promising discriminative ability to identify patients with borderline VWF levels who are likely to have VWD.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
ABO blood group system; Bayesian prediction; Blood coagulation; Von willebrand disease; Von willebrand factor
Elenco autori:
P. Bucciarelli, S.M. Siboni, F. Stufano, E. Biguzzi, M.T. Canciani, L. Baronciani, M.T. Pagliari, S. La Marca, C. Mistretta, F.R. Rosendaal, F. Peyvandi
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