Hemophilia B is a recessive X-linked bleeding disorder characterized by deficiency of the coagulation factor IX (FIX). In hemophilia B patients the severity of the bleeding phenotype is related to the degree of the FIX defect. Hemophilia B treatment has improved greatly in the last 20 years with the introduction first of plasma-derived and then of recombinant FIX concentrates. Replacement thera...

The history of hemophilia is ancient, with descriptions dated to the 2nd century AD. first modern narratives appeared in 1800s, when total blood transfusion was only available treatment and life expectancy remarkably low. Advances occurred use plasma cryoprecipitate, but discovered factor concentrates revolutionized treatment. implantation prophylaxis allowed hemophilic patients prevent bleedin...

Up to 30% of haemophilia A patients and up to 3% of haemophilia B patients develop neutralizing antibodies (inhibitors) to factor VIII or factor IX, rendering replacement therapies ineffective and making the management of bleeding episodes particularly challenging.1–3While patients with low titre inhibitors may benefit from higher doses of factors VIII or IX, patients with higher antibody level...

The objectives of this article were to study the reported prevalence of haemophilia B (HB) on a country-by-country basis and to analyse whether the prevalence of HB varied by national economy. The prevalence of HB is the proportion of diagnosed, reported cases of HB in a population at a specific point of time. We collected data on the HB prevalence for 105 countries from the World Federation of...

We used a mouse model to test the hypothesis that the time course and histology of wound healing is altered in hemophilia B. Punch biopsies (3 mm) were placed in the skin of normal mice and mice with hemophilia. The size of the wounds was measured daily until the epidermal defect closed. All wounds closed in mice with hemophilia by 12 days, compared with 10 days in normal animals. Skin from the...