The management of patients with inhibitors is the greatest challenge facing haemophilia health professionals. Immune tolerance induction (ITI) can be successful in eliminating the inhibitor in the majority of CHIR-99021 research buy patients, provided it is started soon after the inhibitor develops and the titre of the inhibitor is <10 BU at commencement of ITI. Acute bleeding is treated using one of two bypassing agents, which exhibit similar efficacy and safety. Surgery in inhibitor patients is challenging and should only be carried out in experienced centres. The use of clotting factor concentrates

has transformed the lives of persons with haemophilia. Unfortunately the use of non-virally inactivated products prior to the mid-1980s resulted in most recipients being infected with hepatitis C and many also with HIV. The use of viral inactivation proved highly efficient in virtually eliminating the infective risk of plasma-derived products. Romidepsin datasheet Furthermore, in the

last 20 years recombinant products, which do not carry the infective risk have been introduced. The most important adverse event associated with factor VIII concentrate use today is the development of FVIII alloantibodies (inhibitors). Inhibitors are more likely to occur during the first 50 exposure days in previously untreated patients (PUPS) and develop in up to a third of the severe patients.

There selleckchem is debate in the literature as to whether there is a difference in the inhibitor risk in PUPS between plasma derived and recombinant concentrates [1], and a randomized clinical trial is currently investigating this. Inhibitors in previously treated patients are much rarer, occurring in approximately 2 per 1 000 patient years and recently there has been a debate as to whether B-domain deleted FVIII concentrates are associated with a higher inhibitor risk than full-length products [2,3]. Once an inhibitor develops, it results in increased mortality, morbidity and cost for the affected individual [4]. It is sometimes possible to eliminate the inhibitor using immune tolerance induction where FVIII is administered regularly and frequently. When bleeding occurs in the presence of an alloantibody, treatment with a bypassing agent is required. Sometimes it is necessary to carry out emergency or elective surgery in the presence of an inhibitor and this can be very challenging. In this manuscript the issues of ITI, treatment with bypassing agents and surgery in patients with inhibitors are reviewed. Bypassing agents are less effective for treatment of bleeding and only partially effective as prophylaxis in inhibitor patients, compared with FVIII in non-inhibitor patients.