rFVIIa

Acquired hemophilia A (AHA) is a rare, life-threatening hemorrhagic disease caused by autoantibodies against factor VIII (FVIII), and bypassing agents (BPA) are used to control bleeding. However, some cases need a change of BPA or BPAs given sequentially or in combination for refractory bleeding. A 71-year-old man was admitted with subcutaneous hemorrhage. Laboratory investigations showed prolongation of activated partial thromboplastin time (APTT) and low-coagulation FVIII activity and FVIII inhibitor; we, therefore, diagnosed AHA...

UNLABELLED: Reports describing symptoms and treatment of patients with congenital factor VII (FVII) deficiency frequently relate to patients in Europe, while only a small number describe data from Asian countries.This multicenter, prospective observational study (NCT01312636) collected data from 30 sites for 55% of patients registered in 2011 in Japan with congenital FVII deficiency treated with activated recombinant FVII (rFVIIa) for bleeding episodes and/or during surgery.The mean follow-up in 20 eligible patients was 11 months (range 1-49 months)...

rFVIIa, a human recombinant activated coagulation factor VII, has been used worldwide for more than two decades for the treatment of bleeding episodes and prevention of bleeding in patients undergoing surgery/invasive procedures with congenital haemophilia A or B with inhibitors (CHwI A or B), acquired haemophilia (AH), congenital factor VII deficiency and Glanzmann thrombasthenia (GT), refractory to platelet transfusion. The approved dosage, administration and indication of rFVIIa in the US, Europe and Japan differ, depending on the needs of the patient population and regulatory practices...

Introduction: Acquired factor V inhibitor (AFVI) is a rare autoimmune bleeding disorder. The treatment of AFVI is challenging, and patients often require both bleeding control and inhibitor eradication. Methods: We conducted a retrospective analysis of the medical records of a 35-year-old Caucasian woman who presented with severe AFVI-induced bleeding and subsequent immunosuppressive therapy. Results: To provide haemostasis, rFVIIa was given with good efficacy. The patient was treated with various combinations of immunosuppressive regimens over the course of 2...

Thrombotic events are a rare complication of recombinant activated factor VII (rFVIIa) therapy in patients with hemophilia. We present a case of a 71-year-old male who developed ST-elevation myocardial infarction after receiving rFVIIa replacement therapy for acquired hemophilia A.

Acquired hemophilia A (AHA) is a rare hemorrhagic coagulopathy caused by the presence of autoantibodies that inhibit the activity of factor VIII (FVIII). Its diagnosis requires a high index of suspicion. It should be suspected in the presence of extensive hematomas or intense mucosal bleeding in patients with no history of previous trauma or hemorrhagic symptoms. We present two clinical cases of AHA, with different presentations and therapeutic management based on immunosuppression and hemostatic control through bypass agents such as activated recombinant FVII (rFVIIa; Novoseven®) and activated prothrombin complex concentrate (aPCC; Feiba®)...

Uncontrolled bleeding is a life-threatening emergency that requires immediate intervention. Currently available on-site bleeding interventions largely rely on the use of tourniquets, pressure dressing, and other topical hemostatic agents, which can only treat bleeding injuries that are known, accessible, and potentially compressible. Synthetic hemostats that are stable at room temperature, easy to carry, field-usable, and able to stop internal bleeding at multiple or unknown sources, are still lacking. We recently developed a hemostatic agent via polymer peptide interfusion (HAPPI), which can selectively bind to activated platelets and injury sites after intravascular administration...

BACKGROUND: Hemostasis in critically ill patients represents a fragile balance between hypocoagulation and hypercoagulation, and is influenced by various factors. Perioperative use of extracorporeal membrane oxygenation (ECMO)-increasingly used in lung transplantation-further destabilizes this balance, not least due to systemic anticoagulation. In the case of massive hemorrhage, guidelines recommend considering recombinant activated Factor VII (rFVIIa) as an ultima ratio treatment only after several preconditions of hemostasis have been established...

Emicizumab is a humanized recombinant bispecific antibody, bridging together activated factor IX (FIXa) and factor X (FX), thus mimicking the activity of FVIII in vivo. Emicizumab is designed for long-term prophylaxis in patients with severe hemophilia A with and without inhibitors. This approach provides constant protection, with significant reduction in bleeding rate and improved quality of life. However, protection provided by emicizumab is not absolute, and clotting factor concentrates (FVIII, rFVIIa, aPCC) may be necessary for post-traumatic bleeding or surgery, with a potential thrombotic risk or difficulty in preventing bleeding...

OBJECTIVE: This study aims to compare the efficacy and safety of activated recombinant factor VII (rFVIIa) and prothrombin complex concentrate (PCC) in the treatment of bleeding complications following surgery requiring cardiopulmonary bypass (CPB) in children. DESIGN/METHODS: This is a retrospective chart review of a single institution comprising patients aged 0 to 18 years old with congenital heart disease. Patients must have received either PCC or rFVIIa after coming off CPB...

OBJECTIVES: To report the perioperative management experience of central venous access devices (CVAD) in Chinese children with severe hemophilia A (SHA) in China. METHODS: This retrospective study included SHA children who underwent Port-A-Cath or peripherally inserted central catheter (PICC) implantation between 2020/01 and 2021/07. Collected data included baseline characteristics, factor replacement regimen and CVAD-related complications. RESULTS: Nine patients had nine ports placed, and eight patients underwent 10 PICCs placement...

OBJECTIVE: To describe the clinical presentation, clinical course, and successful management of noncompressible, abdominal hemorrhage with recombinant human factor VIIa (rFVIIa) in 2 postoperative patients. CASE SUMMARY: A 14-year-old neutered female Border Terrier and a 9-year-old neutered male domestic shorthair were treated with rFVIIa to treat noncompressible abdominal hemorrhage in the postoperative period. The dog presented for a septic abdomen following endoscopic intestinal biopsies 10 days prior and was found to have a jejunal perforation along with a fractured liver lobe and hepatic lymphoma at the time of exploratory laparotomy...

An 86-year-old Japanese male patient visited a nearby hospital with painful swelling in his left upper and lower limbs 35 days after the second dose of the BNT162b2 mRNA coronavirus disease-2019 (COVID-19) vaccine. He was referred to our hematological department due to a prolonged activated partial thromboplastin time and was urgently admitted. He was diagnosed with acquired hemophilia A (AHA) based on factor VIII (FVIII) activity of 1.7%, FVIII inhibitor of 152.3 BU/ml, and FVIII-binding antibodies detected by enzyme-linked immunosorbent assay...

BACKGROUND: In the SPOTLIGHT trial (Spot Sign Selection of Intracerebral Hemorrhage to Guide Hemostatic Therapy), patients with a computed tomography (CT) angiography spot-sign positive acute intracerebral hemorrhage were randomized to rFVIIa (recombinant activated factor VIIa; 80 μg/kg) or placebo within 6 hours of onset, aiming to limit hematoma expansion. Administration of rFVIIa did not significantly reduce hematoma expansion. In this prespecified analysis, we aimed to investigate the impact of delays from baseline imaging to study drug administration on hematoma expansion...

Factor XI (FXI) deficiency is an autosomal bleeding disorder characterized by injury-related hemorrhage, mostly associated with surgical procedures at sites noted for high fibrinolytic activity. Severe FXI deficiency is defined when the FXI level is lower than 15-20 IU/dL. Perioperative prophylactic treatment for high-bleeding-risk surgery in patients with severe FXI deficiency is based on fresh frozen plasma (FFP) transfusions or FXI concentrate (where available). Exposure to FFP and to FXI concentrate may lead to the development of inhibitory antibodies against FXI...

BACKGROUND: Preclinical bleeding models increase current hemophilia A (HA) knowledge and aid the development of new pharmacological treatments. There are several well-established mouse bleeding models, but limited options are available for rat models despite their high resemblance to human disease process. OBJECTIVE: To provide a comprehensive description of the tail vein transection (TVT) bleeding model in HA rats and examine the correlation between in vivo pharmacological efficacy and global hemostatic assays...

GEN-0828, a proposed clinical candidate for hemophilia and trauma hemorrhage treatment, is a novel recombinant activated human factor VII (rFVIIa). The purpose of this paper is to compare the pharmacokinetics and pharmacodynamics of GEN-0828 in hemophilia B mice with those of NovoSeven®, the only marketed rFVIIa product worldwide., GEN-0828 and NovoSeven® showed similar affinity bioactivity to recombinant tissue factor (rTF) in vitro. Pharmacodynamics data indicated a generally similar hemostatic efficacy (ED50 ) of GEN-0828 (10...

OBJECTIVE: To evaluate the financial impact of utilizing rpFVIII or rFVIIa during a hospital admission for the diagnosis of acquired hemophilia A (AHA) by reviewing the margin between the cost to the hospital for providing care and the amount the hospital is reimbursed by the Centers for Medicare & Medicaid Services (CMS) in the US. METHODS: Data source was the Medicare Limited Data Set, which contains claims for hospitalizations, charges, and amounts reimbursed by CMS...

Recombinant factor VIIa (rFVIIa) has of April 2022 been approved by the European Medicines Agency for treatment of severe postpartum haemorrhage. The extended approval is based on one small open-label, non-blinded randomized trial of 84 women from 2015 showing reduced "second line" treatment, but also increased risk of thromboembolism. Systematic use of tranexamic acid and timely coagulation assessment with thromboelastography/rotational thromboelastometry are not applied. Danish and international societies recommend that rFVIIa is reserved as a last option in severe life-threatening cases of postpartum haemorrhage...

BACKGROUND: Coagulopathy following cardiac surgery is associated with considerable blood product transfusion and high morbidity and mortality. The treatment of coagulopathy following cardiac surgery is challenging, with the replacement of clotting factors being based on transfusion of fresh frozen plasma (FFP). Prothrombin complex concentrate (PCCs) is an alternative method to replace clotting factors and warrants evaluation. PCCs are also an alternative method to treat refractory ongoing bleeding post-cardiac surgery compared to recombinant factor VIIa (rFVIIa) and also warrants evaluation...