BACKGROUND: Quantitative bleeding assessment tools (BATs) have been used to describe the severity of the bleeding phenotype in patients with von Willebrand disease. OBJECTIVES: To evaluate the clinical usefulness of a BAT for the diagnosis of mild bleeding disorders (MBDs) in previously undiagnosed patients. METHODS: We prospectively assessed 215 patients who were consecutively referred for evaluation of bleeding symptoms (n=71), abnormal laboratory clotting test results (n=105) or family investigation (n=39) at two second-level centers. The bleeding history was assessed by a young investigator who administered the BAT instrument, and also by a senior physician who independently evaluated the patient and made the final diagnoses. Sensitivity, specificity, negative predictive value (NPV) and positive predictive value (PPV) were computed for a predefined bleeding score (BS) cut-off (BS of >3). Receiver operating characteristic curves were used to establish a diagnostic prediction rule. RESULTS: Assuming the prevalence of MBD in the general population to be ∼1%, a normal BS (≤3) had a very high NPV (99.2%). The PPVs in patients referred for hemostatic or family evaluation at second-level clinics were estimated to be 71.0% and 77.5% (assuming MDB prevalences of 20% and 50%, respectively, in these settings). Measurement of BS in addition to activated partial thromboplastin time significantly increased the diagnostic efficiency of the BAT instrument (NPV of 99.6%). CONCLUSIONS: BAT use improves the evaluation of patients with suspected MBD, and we propose its use in a clinical prediction guide based on BAT and activated partial thromboplastin time for the exclusion of patients with suspected MBD in a low-prevalence setting.
BACKGROUND: Quantitative bleeding assessment tools (BATs) have been used to describe the severity of the bleeding phenotype in patients with von Willebrand disease. OBJECTIVES: To evaluate the clinical usefulness of a BAT for the diagnosis of mild bleeding disorders (MBDs) in previously undiagnosed patients. METHODS: We prospectively assessed 215 patients who were consecutively referred for evaluation of bleeding symptoms (n=71), abnormal laboratory clotting test results (n=105) or family investigation (n=39) at two second-level centers. The bleeding history was assessed by a young investigator who administered the BAT instrument, and also by a senior physician who independently evaluated the patient and made the final diagnoses. Sensitivity, specificity, negative predictive value (NPV) and positive predictive value (PPV) were computed for a predefined bleeding score (BS) cut-off (BS of >3). Receiver operating characteristic curves were used to establish a diagnostic prediction rule. RESULTS: Assuming the prevalence of MBD in the general population to be ∼1%, a normal BS (≤3) had a very high NPV (99.2%). The PPVs in patients referred for hemostatic or family evaluation at second-level clinics were estimated to be 71.0% and 77.5% (assuming MDB prevalences of 20% and 50%, respectively, in these settings). Measurement of BS in addition to activated partial thromboplastin time significantly increased the diagnostic efficiency of the BAT instrument (NPV of 99.6%). CONCLUSIONS: BAT use improves the evaluation of patients with suspected MBD, and we propose its use in a clinical prediction guide based on BAT and activated partial thromboplastin time for the exclusion of patients with suspected MBD in a low-prevalence setting.
Authors: Giuseppe Tagariello; Paolo Radossi; Roberta Salviato; Milena Zardo; Lucia De Valentin; Marco Basso; Giancarlo Castaman Journal: Blood Transfus Date: 2016-07-22 Impact factor: 3.443
Authors: Michelle Lavin; Sonia Aguila; Sonja Schneppenheim; Niall Dalton; Kenneth L Jones; Jamie M O'Sullivan; Niamh M O'Connell; Kevin Ryan; Barry White; Mary Byrne; Marie Rafferty; Mairead M Doyle; Margaret Nolan; Roger J S Preston; Ulrich Budde; Paula James; Jorge Di Paola; James S O'Donnell Journal: Blood Date: 2017-09-15 Impact factor: 22.113