Clinical Study of Recombinant Human Activated Coagulation Factor VII for Injection in Patients With Hemophilia With Inhibitor

Inclusion Criteria:

• 1 Diagnosed as congenital hemophilia A or B, and meet the following conditions:

1. FⅧ<1% or FIX activity<2%;
2. FⅧ inhibitor or FⅨ inhibitor titer in the screening period>5 BU (Nijmegenmodified Bethesda method of detection)."

• 2 Age ≥18 and ≤65 years, male or female.
• 3 There have been at least two incidents of bleeding of any kind in the last sixmonths.
• 4 No other drugs for the treatment of hemophilia have been used within 72hours (3days) before administration, including prothrombin complex and any coagulation factorVII or activated coagulation factor VII、coagulation factor VIII、coagulation factor IXproducts, cryoprecipitate, fresh plasma and whole blood, etc.
• 5 Subjects of childbearing age agree to take effective contraceptive measuresthroughout the trial period, and continue to 28 days after the last medication.
• 6 Volunteer to participate in this study, sign an informed consent form, have goodcompliance, and be able to cooperate with the experimental observation.

Exclusion Criteria:

• 1 Any other bleeding disease except Congenital hemophilia A or B.
• 2 Patients with any previous medical history or symptoms of arterial or venousthromboembolic events (such as atherosclerosis, myocardial infarction, ischemicstroke, transient ischemic attack, deep vein thrombosis or pulmonary hypertensionembolism) or disseminated intravascular coagulation (DIC) within the past 1 year.
• 3 Baseline and previous values of FⅦ inhibitor or activated recombinant humancoagulation factor VII inhibitor is positive.
• 4 Vitamin K deficiency.
• 5 Human immunodeficiency virus (HIV) positive and cluster of differentiation 4 (CD4)count ≤200/μl, the number of virus carriers ≥200 particles/μl or ≥400000 copies/ml.
• 6 Subjects plan to perform elective surgery during the trial period.
• 7 Those who are allergic to test drugs or any excipients.
• 8 Severe anemia and need blood transfusion.
• 9 Platelet count <80×10^9/L.
• 10 Obvious liver or kidney damage: glutamic-pyruvic transaminase (ALT) or aspartictransaminase (AST)>2.5×ULN, or total bilirubin>1.5×ULN or serum creatinine>1.5×ULN.
• 11 Severe heart disease, including myocardial infarction, cardiac insufficiency grade 3 or above, the current New York Heart Association cardiac function grade II-IV.
• 12 There was a prior intracranial hemorrhage
• 13 Those who had used or planned to use any anticoagulants, antifibrinants and drugsaffecting platelet function during the first week of medication included non-steroidalanti-inflammatory drugs (NSAIDs) such as aspirin
• 14 Hypertension that cannot be controlled with drug treatment: systolic bloodpressure> 150 mmHg or diastolic blood pressure> 90 mmHg.
• 15 Participated in other clinical studies (except Activated coagulation factor VII,coagulation factor VIII and coagulation factor IX trials) within one month before thefirst medication.
• 16 Alcoholism, drug abuse, mental disorders, other severe acute or chronic diseases,greater abnormal laboratory values, and those who are considered unsuitable by theresearcher.