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Large psoas haematoma and femoral neuropathy in a patient with type 2B von Willebrand's disease

replacement was started within 1 h, whereas almost half (48.9%) of the cohort was treated with a 1–12 h delay. Several reasons, such as transport problems, inadequate factor concentrate supply at hospitals or non-awareness of medical staff for appropriate and timely treatment in PWH could have contributed to these delays. Furthermore, ignorance of patients and parents might have added to the problem. In four cases where patients died due to head injury, the GCS was not recorded and in nine cases of death due to ICH, no vital parameters were documented in the records. For the stab victim, no GCS or vital parameters were recorded and for the gunshot victim, only vital parameters were available (injury to chest and abdomen only). The lack of documentation of these important parameters indicates the non-awareness of the importance of proper initial monitoring of PWH in this study population and may have contributed to the overall mortality rate of 18.6%. Interestingly, of the 40 patients on home therapy, only three patients self-administered factor concentrate and two were treated at peripheral hospitals prior to their admission to a Haemophilia Comprehensive Care Centre. No patient in this cohort received primary prophylaxis and only 10.5% received secondary prophylaxis. None of the 16 patients who died received prophylaxis and only five were on home therapy. Although these numbers are small and conclusions cannot be drawn from this data, it is expected that a patient on prophylaxis, would have a better outcome in the case of a traumatic incident. Nine per cent of PWH developed potentially avoidable sequelae such as flexion deformities, chronic synovitis or hemiparesis arising from bleeding events. This study confirms the importance of timely treatment of PWH who have substantial risks for suffering trauma-related complications. Time delays in starting treatment might have contributed to the mortality rate seen in this cohort. The study also reinforces the fact that preventative strategies such as home therapy or regular haemophilia clinic visits improve survival in PWH with trauma-related bleeding events. Hence, education of ambulance, primary clinics and ED staff remains paramount to successful treatment outcomes. Finally, education of patients and their parents should emphasize the importance of early treatment and regular clinic attendance.