A prospective study involving larger numbers of PUPS with severe haemophilia A is required to assess more extensively the potential benefits of a once weekly early prophylaxis scheme. PM Mannucci, Q Shi, S Bonanad and R Klamroth received an selleck kinase inhibitor honorarium from Grifols S.A. for participating in the symposium and production of the article. The authors thank Content Ed Net for providing editorial assistance in the preparation of the article, with funding by Grifols

S.A. “
“From a young age patients with severe and moderately severe FIX deficiency (haemophilia B) can experience spontaneous or traumatic bleeding and joint destruction may result. The use of coagulation factor IX concentrate to prevent anticipated bleeding, as primary or secondary prophylaxis, has become a FK506 ic50 common and recommended practice in children. The current practice of using tertiary prophylaxis, in

the presence of established joint arthropathy, in adults with haemophilia B is not well characterized. This observational study was conducted to gain a better understanding of the recent Canadian experience with tertiary prophylaxis in adults with severe and moderately severe haemophilia B. Data were collected from all eligible adult (≥ 18 years of age) males with baseline FIX:C ≤ 2% from seven Canadian Hemophilia Treatment centres over a 2-year observation period from 2009 to 2011. Thirty-four per cent of the 67 subjects with moderately severe haemophilia B were exposed to prophylaxis with the majority as continuous prophylaxis (≥45 weeks year-1). The severe subgroup (FIX:C < 1%) demonstrated a 52% exposure rate. None had primary prophylaxis exposure in childhood. Eighty-one per cent used once or twice MCE weekly infusion regimens

and reported a median annual bleeding rate of five bleeds per year versus four bleeds per year for those using on-demand treatment. Annual median factor utilization for all subjects using prophylaxis was 196 283 U year-1 compared to 46 361 U year-1 for on demand. Approximately 50% of adults with severe haemophilia B are using continuous tertiary prophylaxis in Canada, a practice likely to increase which warrants further study. “
“Summary.  Menorrhagia is the most common bleeding manifestation in women with inherited bleeding disorders. There is little known about whether the management of menorrhagia is altered in specific bleeding disorders. Optimizing treatment strategies for each specific diagnosis may improve quality of life in these women. This work aimed to look for a potential relationship between the specific diagnosis of an inherited bleeding disorder and the intervention required to control the menorrhagia.