Inhibitor incidence in an unselected cohort of previously untreated patients with severe haemophilia B: a PedNet study.

ABSTRACT: The incidence of FIX inhibitors in severe hemophilia B (SHB) is not well defined. Frequencies of 3-5% have been reported but most studies to date were small, including patients with different severities, and without prospective follow-up for inhibitor incidence. Study objective was to investigate inhibitor incidence in patients with SHB followed up to 500 exposure days (ED), the frequency of allergic reactions, and the relationship with genotypes. Consecutive previously untreated patients (PUPs) with SHB enrolled into the PedNet cohort were included. Detailed data was collected for the first 50 ED, followed by annual collection of inhibitor status and allergic reactions. Presence of inhibitors was defined by at least two consecutive positive samples. Additionally, data on factor IX gene mu

SUBMITTER: Male C 

PROVIDER: S-EPMC7776246 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature