Introduction Joint bleeding is still a challenging complication of hemophilia. Distinguishing between intra-articular hemarthrosis and peri-articular soft tissue hematomas appears important but little guidance exist to support self-diagnosis by patients in the home care setting.

Introduction The A-MORE study primarily aims to evaluate the long-term effectiveness of efmoroctocog alfa (referred to herein as rFVIIIFc) on joint health in patients with haemophilia A (PwHA) in a real world setting.

Objective Acquired von Willebrand Syndrome (avWS) is a rare coagulation disorder which can be associated with IgM-paraproteinaemia. Some patients develop specific antibodies against von Willebrand factor (vWF) that increase its clearance. Recently, recombinant vWF has become available for the treatment of bleedings and prevention of surgical bleedings in patients with inherited von Willebrand’s disease, but experience in patients with avWS is limited.

[This corrects the article DOI: 10.1371/journal.pgen.1008690.].

Objective Formation of anti-FVIII antibodies (inhibitors, Inh) is a severe complication of FVIII substitution therapy in haemophilia A. Inh can bind to FVIII thereby forming FVIII-IC with possibly harmful consequences like enhanced FVIII uptake by antigen-presenting cells in ongoing anti-FVIII immune reaction. There is a controversial debate about potential immuno-protective effects of vWF in regard to Inh development, but data about vWF impact on subsequent FVIII-IC formation are not available. This study is focused on the vWF influence on FVIII-IC characteristics using analytical ultracentrifugation (AUC).

Objective Acquired hemophilia A (AHA) is an extremely rare, but potentially life-threatening bleeding disorder caused by autoantibodies against human coagulation factor VIII. AHA may lead to spontaneous or trauma induced bleeds, treated with bypassing agents. New and successful therapies had been launched in the last decade. Several manufacturers drove awareness campaigns in Germany to push detection of potential patients. Here we examine the change in frequency of AHA in-hospital coding, based German DRG-data.

Objectives: Anti-factor VIII (FVIII) antibodies (Ab) neutralize the hemostatic activity of FVIII and modulate anti-FVIII immune responses via the formation of FVIII-containing immune complexes (FVIII-IC). In the circulation, vWF protects FVIII from proteolytic cleavage by proteinases and competes with anti-FVIII Ab for FVIII binding sites. In this study, we investigated the impact of vWF to influence FVIII-IC formation and thereby affecting FVIII-specific secondary immune response in hemophilia A (HA) mouse model.

Scientific Research Question: Recombinant factor VIII Fc (rFVIIIFc) and recombinant factor IX Fc (rFIXFc) have been approved on the basis of their pivotal phase 3 trials in children, adolescents and adults with haemophilia A or B, respectively. The ongoing PREVENT study (NCT03055611) is a non-interventional study with the objective to provide data on the real-world usage and effectiveness of prophylaxis with rFVIIIFc and rFIXFc in Germany over a 24-month prospective period. Here the aim is to report on the current status of the study and present baseline characteristics and retrospective clinical data.