The predictive value of bleeding score on the diagnosis of Von Willebrand disease in children applied to the hematologic clinic with epistaxis

Abstract

Aim: Epistaxis may be a symptom of an inherited bleeding disease.. We aimed to analyze an approved pediatric bleeding score (PBS) as a screening test for von Willebrand Disease (VWD) in children with epistaxis Material and Method: We retrospectively reviewed the medical records of pediatric patients, who applied to the Pediatric Hematology Department with the complaint of epistaxis between January 2018 and December 2019. Results: One hundred and sixty eight patients enrolled in this study There were 65(38.7%) girls and 103(61.3%) boys, with a mean age of 114±49 months (range 8 months to 18 years).The PBS of 34 patients was greater than/ or equal to 2. Factor 8, von Willebrand factor antigen, and von Willebrand Ristocetin cofactor levels were significantly lower in patients with PBS≥2 compared to those in patients with PBS<2 (%73±43 vs % 91±29, p=0.03; 87±44 vs 106±29 IU/dl, p=0.03; 72±39 vs 98±30 IU/ dl, p=0.001, respectively). While 15 (44%) of 34 patients with PBS ≥ 2 diagnosed VWD, but in the group with PBS<2, VWD was diagnosed for only 4 children (0.02%) (4/134). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of PBS for diagnosis of VWD was 79.0%, 87.2%, 44%, and 97% respectively. Conclusion: PBS could be integrated into the evaluation of children suspected of having a bleeding disorder such as VWD in pediatrician's offices. Our cut off value 2 appears to be significant in exclusion of VWD, since its high negative predictive value.