Evaluation of the utility of the ISTH‐BAT in haemophilia carriers: a multinational study

• DOI: 10.1111/hae.13089
• Publication Date: 2016-11-21T08:55:38Z
• Authors: P. D. James; J. Mahlangu; C. Bidlingmaier; M. E. Mingot‐Cast...; M. Chitlur; P. F. Fogarty; A. Cuker; M. E. Mancuso; P. A. Holme; J. Grabell; N. Satkunam; W. M. Hopman; P. Mathew
• Keywords: Haemophilia B

Introduction There has been increasing recognition in recent years that female carriers of haemophilia manifest abnormal bleeding; however, data on the use bleeding assessment tools this population are lacking. Aim Our objective was to validate ISTH ‐ BAT describe symptoms and allow for comparisons with factor levels other patient groups. Methods This a prospective, observational, cross‐sectional study performed by members Global Emerging HE mostasis Panel ( GEHEP ). Unselected consecutive were recruited CRF completed personnel. Results A total 168 enrolled: 155 13 B. The mean age 40 (range: 20–82). Carriers had higher scores BS ) compared age‐matched controls n = 46; 5.7 vs. 1.43; P < 0.0001) Type 3 VWD OC 32; 3.0; 0.009), but lower women 1 83; 8.7; 0.0001). Fifteen reported haemarthrosis, those six normal FVIII / FIX levels. significant weak negative correlation between level (Spearman's r 2 −0.36, 0.001). Conclusion results show experience bleeding, including haemarthrosis. Overall, > controls. Understanding performance is critical step future research aimed at investigating underlying pathophysiology ultimate goal optimizing treatment.