In the absence of evidence-based treatment guidelines,

Ibrutinib this article presents 10 cases of difficult to control acute and surgical bleeding and offers consensus opinions regarding their management from a panel of experienced haemophilia treaters. “
“Congenital haemophilia is a rare and complex condition for which dedicated specialized and comprehensive care has produced measurable improvements in clinical outcomes and advances in patient management. Among these advances is the ability to safely perform surgery in patients with inhibitor antibodies to factors VIII and IX, in whom all but the most necessary of surgeries were once avoided due to the risk for uncontrollable Rucaparib molecular weight bleeding due to ineffectiveness of replacement therapy. Nevertheless, surgery continues to pose a major challenge in this relatively rare group of patients because of significantly higher costs than in patients without inhibitors, as well as a high risk for bleeding and other complications. Because of the concentration of expertise and experience, it is recommended that any surgery in patients with haemophilia and inhibitors be planned in conjunction

with a haemophilia treatment centre (HTC) and performed in a hospital that incorporates a HTC. Coordinated, standard pre-, intra- and postoperative assessments and planning are intended to optimize surgical outcome and utilization of resources, including costly factor concentrates and other haemostatic agents, while minimizing the risk for bleeding and other adverse consequences both during and after surgery. This article will review the special considerations for patients with inhibitors as they prepare for and move through surgery and recovery, with an emphasis on the roles and responsibilities of individual members of the multidisciplinary

team in facilitating this process. Congenital haemophilia, a rare and complex condition, requires a lifetime of specialized care. A network of haemophilia treatment centres (HTCs) has been established in many developed countries to provide dedicated comprehensive, 上海皓元医药股份有限公司 multidisciplinary care in a single setting [1]. In the United States, the provision of haemophilia care by these centres has led to an array of documented improved outcomes, including substantial reductions in hospital visits, health care costs, work and school absenteeism and even mortality [2, 3]. Even in developing countries with limited haemostatic treatment options, the establishment of local expertise via such initiatives as ‘twinning’ programmes has resulted in improvements in patient care [1]. Approximately 20–30% and 1–6% of patients with severe haemophilia A and B, respectively [4], develop inhibitory antibodies that render replacement therapy ineffective, potentially leading to life-threatening bleeding events.