Quantitation of Bleeding Symptoms in Children with von Willebrand Disease (VWD) or a Platelet Function Disorder: Use of a Standardized Pediatric Bleeding Score.
Authors: Biss T.T. , Blanchette V.S. , Bowman M. , Clark D.S. , Silva M. , Lillicrap D. , James P.D. , Rand Margaret L. 
Published: April 2020

Obtaining an accurate bleeding history is an essential step in the diagnosis of a bleeding disorder, which is often made during childhood. A bleeding history is usually taken in an informal manner, with the interpretation of the responses dependent on the experience of the observer. The development of standardized bleeding questionnaires has improved objectivity and allowed the determination of quantitative scores according to the severity of mucocutaneous bleeding. In adults, using the International Society on Thrombosis and Haemostasis (ISTH) Bleeding Questionnaire for Diagnosis of Type 1 VWD, a bleeding score of &gt;3 in males and &gt;5 in females is considered abnormal (Rodeghiero et al., J Thromb Haemost2005:3;2619). Children have often not been exposed to hemostatic challenges and may have low scores despite significant bleeding disorders. Symptoms specific to childhood, such as post-circumcision bleeding, umbilical stump bleeding and cephalohematoma may be of greater significance in this patient group. Thus, we adapted the ISTH Bleeding Questionnaire for use in pediatrics, with a symptom-specific grade of -1 to 4 (Tosetto et al., J Thromb Haemost2006:4;766). Bleeding scores were determined by interview of parents/children for 80 children with a previous diagnosis of VWD or a platelet function disorder at The Hospital for Sick Children, Toronto, or Kingston General Hospital, Kingston. 62 children had a diagnosis of VWD and 18, a disorder of platelet function (Glanzmann thrombasthenia: 3; dense granule defect: 2; MYH9-related thrombocytopenia: 2; Hermansky-Pudlak syndrome: 1; unspecified: 10). 45 children were female and 35, male (median age: 10 yrs (range: 9 mo-17 yrs)), with a median age of females of 12 yrs (range: 0–17) and of males, 9 yrs (range: 1–17). Bleeding scores ranged from 0–28 (median: 7), with a median score in females of 7 and in males, 8. Bleeding scores according to diagnosis and age are shown in Tables 1 and 2, respectively. The most frequent reasons for a positive score of ≥2 were epistaxis (43% of patients), bleeding from minor wounds (38%), bleeding after dental extraction (31%) and excessive bruising (26%). Menorrhagia requiring treatment occurred in 47% of menstruating females. Bleeding from the umbilical stump, post-circumcision bleeding and cephalohematoma were reported in 10%, 6% and 4% of patients, respectively. In summary, we have used a standardized bleeding questionnaire, adapted for use in pediatrics, with an accompanying score to quantify bleeding symptoms in children with confirmed VWD or a platelet function disorder. Bleeding scores were lowest in the youngest age group (0–3 yrs), and were slightly higher in males than in females. Bleeding occurred early in childhood, i.e. post-circumcision bleeding and bleeding from the umbilical stump. This standardized pediatric bleeding questionnaire/score may be useful in clinical practice in the assessment of children presenting with symptomatic bruising and bleeding.

Diagnosis Type 1 VWD Type 2 VWD Type 3 VWD Platelet function disorder Number of patients 48 8 6 18 Median bleeding score (range) 6 (0–18) 10 (0–17) 14 (9–28) 9 (1–19) Age (years) 0–3 4–6 7–9 10–12 13–15 16–18 Number of patients 7 13 18 16 14 12 Median bleeding score (range) 2 (0–9) 10 (0–16) 6 (0–19) 9.5 (1–28) 6 (2–18) 12 (6–21)