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

Yıl 2022, Cilt: 5 Sayı: 1, 22 - 25, 17.01.2022

Ayşegül Alpcan , Serkan Tursun , Yaşar Kandur , Mustafa Çağlar Yörgüç , Meryem Albayrak

https://doi.org/10.32322/jhsm.984860

Öz

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.

Anahtar Kelimeler

Pediatric bleeding score, Epistaxis, Von Willebrand Disease

Kaynakça

• Sandoval C, Dong S, Visintainer P, Ozkaynak MF, Jayabose S. Clinical and laboratory features of 178 children with recurrent epistaxis. J Pediatr Hematol Oncol 2002; 47-9.
• Recht M, Chitlur M, Lam D. Epistaxis as a common presenting symptom of Glanzmann’s thrombasthenia, a rare qualitative platelet disorder: illustrative case examples. Case Rep Emerg Med 2017; 2017: 8796425.
• Natalia Rydz , Paula D James. The evolution and value of bleeding assessment tools. J Thromb Haemost 2012; 10: 2223-9.
• Sarah H O'Brien. Bleeding scores: are they really useful? Hematology Am Soc Hematol Educ Program 2012; 2012: 152-6.
• Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD. Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost 2009; 7: 1418–21.
• Bui J, Martyres D, James PD, et al. Validation of the school age self-administered pediatric bleeding questionnaire (Self-PBQ) in children aged 8-12 years. Pediatr Blood Cancer 2019; 66:e27709
• Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-73.
• Rojnuckarin P, Akkawat B, Intragumtornchai T. Von Willebrand factor (VWF) antigen levels and function in healthy Thais. Southeast Asian J Trop Med Public Health 2005; 36: 1292-7.
• Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J. Letter: a method for assaying von Willebrand factor (ristocetin cofactor). Thromb Diath Haemorrh 1975; 34: 306-8.
• Kitchen S, Gray E, Mertens K. Monitoring of modified factor VIII and IX products. Haemophilia 2014; 20: 36-42.
• Nichols WL, Hultin MB, James AH, et al. Von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel Report (USA). Haemophilia 2008; 14: 171-232.
• Leebeek F, Eikenboom J. Von Willebrand’s disease. N Engl J Med 2016; 375: 2067-80.
• James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv 2021; 5: 280-300.
• Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-73.
• Tosetto A, Castaman G, Rodeghiero F. Bleeding scores in inherited bleeding disorders: clinical or research tools? Haemophilia 2008; 14: 415-22.
• Biss TT, Blanchette VS, Bowman M, et al. Quantification of bleeding symptoms in children with von Willebrand disease or a platelet function disorder: use of a standardized pediatric bleeding score. Blood 2007; 110: 636a.
• Belen B, Kocak U, Isik M, et al. Evaluation of pediatric bleeding questionnaire in Turkish children with Von Willebrand disease and platelet function disorders. Clin Appl Thromb Hemost 2015; 21: 565-9.
• Malec LM, Moore CG. Validation study of the composite score to identify Von Willebrand disease in children. J Pediatr Hematol Oncol 2016; 38: 139-42.