Bleeding Phenotype in Children with Moderate or Severe Von Willebrand Disease – Iatrogenic bleeding is the presenting symptom in 42% of the index cases

s ingediend voor het Amsterdam Kindersymposium 2013 101 Bleeding Phenotype in Children with Moderate or Severe Von Willebrand Disease – Iatrogenic bleeding is the presenting symptom in 42% of the index cases Yvonne Sanders (1), Karin Fijnvandraat (2), Evelien Mauser-Bunschoten (3), Anske van der Bom (4), Manon Degenaar-Dujardin (5), Jeroen Eikenboom (6), Britta Laros-van Gorkom (7), Karina Meijer (8), Marjon Cnossen (9), Frank Leebeek (1) (1) Department of Hematology, Erasmus MC, Rotterdam (2) Department of Pediatric Hematology, Emma Children’s Hospital, AMC (3) Van Creveldkliniek and Department of Hematology, UMC Utrecht (4) Department of Clinical Epidemiology, LUMC, Sanquin Research, Leiden (5) Netherlands Hemophilia Society (NVHP), Badhoevedorp (6) Department of Thrombosis & Hemostasis, Einthoven Laboratory, LUMC, Leiden (7) Department of Hematology, UMC St Radboud Nijmegen (8) Department of Hematology, UMCG, Groningen. (9) Department of Pediatric Hematology, Erasmus MC/Sophia Children’s Hospital, Rotterdam. INTRODUCTION Clinical expression of von Willebrand disease (VWD) in children may be diff erent than in adults. Currently there is limited information available on the bleeding phenotype in children with VWD. Therefore we evaluated the occurrence of the type and severity of bleeding symptoms in a large cohort of children with VWD and compared it to adults with VWD. METHODS We included 140 children with type 1 (n=70), 2 (n=51) and 3 (n=19) VWD (VWF levels ≤30 U/dL) and 666 adults (≥ 16 years old) in a nation-wide cross-sectional study (Willebrand in the Netherlands – WiN study). Bleeding severity was determined using the validated adult Tosetto Bleeding Score (BS) with supplementary pediatric-specifi c bleeding symptoms (umbilical stump bleeding, cephalohematoma, post-circumcision bleedings, post-venapuncture bleeding, macroscopic hematuria). RESULTS Median age was 7 years in children and 45 years in adults. In children median BS was 5.5 (IQR 2-9), when pediatric-specifi c bleeding symptoms were included it was 6.0 (IQR 2-10). In adults the median BS was 11.0 (IQR 6-17). Pediatric-specifi c bleeding symptoms were reported in 41 children (29%), occurring in 12 (17%) type 1 VWD, 17 (33%) type 2 VWD and 12 (63%) type 3 VWD patients (p<0.001). Eight out of 31 index cases (26%) presented with a pediatric-specifi c bleeding symptom. An iatrogenic bleeding was the presenting symptom in almost half of the index cases (n=13 out of 31; 42%). The most frequent bleedings in children were: cutaneous, minor wounds, tooth extraction and menorrhagia. Compared to adults with VWD, children had signifi cantly less bleeding from minor wounds, oral cavity and gastro-intestinal tract, after surgery and tooth extraction, muscle hematoma, hemarthrosis and menorrhagia (p<0.05). CONCLUSION Pediatric-specifi c bleeding symptoms occur in a large proportion of children with VWD and iatrogenic bleeding is the presenting symptom in almost half the index case patients.