A Phase 3 Study of the Safety and Efficacy of Coagulation Factor VIIa (Recombinant) for the Prevention of Excessive Bleeding in Patients With Congenital Hemophilia A or B With Inhibitors to Factor VIII or IX Undergoing Elective Major Surgical Procedures SCOPE HIM

Inclusion Criteria:

Each patient must meet the following criteria to be enrolled in this study:

1. be male with a diagnosis of congenital hemophilia A or B of any severity

2. have one of the following:

3. positive inhibitor test BU ≥5 (as confirmed at screening by the institutionallab) OR

4. an inhibitor test BU <5 (as confirmed at screening by the institutional lab)but expected to have an anamnestic response to FVIII or FIX, as demonstrated bya history of a high-responding inhibitor manifested by a previous anamnesticresponse, defined as a peak inhibitor titer >5 BU after re-exposure to factorconcentrates, precluding the use of FVIII or FIX products to treat or preventbleeding OR

5. an inhibitor test BU <5 (as confirmed at screening by the institutional lab)but expected to be refractory to FVIII or FIX, as demonstrated by the patient'shistory of previous failure to respond to FVIII or FIX concentrates, even inthe absence of a documented anamnestic response, precluding the use of FVIII orFIX products to treat or prevent bleeding episodes.

6. be ≥12 years to ≤65 years of age on the day of informed consent

7. be scheduled for an elective major surgical procedure as defined in the studyprotocol (see Table ''Definitions for the specific purpose of Study F7TG2202'')

8. have Hb ≥ 12 g/dL

9. be capable of understanding and willing to comply with the conditions of theprotocol OR in the case of a patient under the age of legal majority,parent(s)/legal guardian(s) must be capable of understanding and willing to complywith the conditions of the protocol

10. have read, understood, and provided written informed consent (patient orparent(s)/legal guardian(s) if the patient is minor according to local regulation)and, where applicable according to local regulation, patient's assent if the patientis minor -

Exclusion Criteria:

Patients who meet any of the following criteria will be excluded from the study.

1. have any coagulation disorder other than hemophilia A or B

2. be immunosuppressed (i.e. the patient should not be receiving systemicimmunosuppressive medication; CD4+ cell counts at screening should be >200/μL)

3. known intolerance to LR769 or any of its excipients

4. currently receiving immune tolerance induction (ITI) therapy

5. have a known or suspected allergy or hypersensitivity to rabbits or rabbit proteins

6. have platelet count <100,000/μL

7. have received an investigational drug within 30 days or within 5 half-lives of thatinvestigational drug, whichever is longer, of the planned first LR769administration, or be expected to receive such drug during participation in thisstudy. Patients who have received fitusiran in a clinical study may not participatein this clinical study for 6 months since the last dose and if they have anantithrombin III level not in the normal range at screening.

8. for patients using emicizumab, have received during the last 6 months or currentlyreceiving a maintenance dosing regimen of emicizumab different from the indicatedone ± 10% of approved dose), i.e. different from 1.5 mg/kg once weekly (±10%), 3mg/kg (±10%) every two weeks or 6 mg/kg (±10%) every four weeks

9. for patients using emicizumab, currently be any plans, or notes in the patient'smedical records that would suggest the need to increase or decrease emicizumabdosing due to antidrug antibodies (ADAs), reduced PK, or coagulation/safety-relatedissues (e.g. lack of response, or potential/actual thromboembolic concerns, etc)

10. have a clinically relevant hepatic (aspartate aminotransferase [AST] and/or alanineaminotransferase [ALT] >3 times the upper limit of normal [ULN]) and/or renalimpairment (creatinine >2 times the ULN)

11. have a history of arterial and/or venous thromboembolic events (such as myocardialinfarction, ischemic strokes, transient ischemic attacks, DVT, or PE) within 2 yearsprior to the planned first dose of LR769, uncontrolled arrhythmia, or current NewYork Heart Association (NYHA) functional classification score of stages II - IV

12. have an active malignancy (those with non-melanoma skin cancer are allowed)

13. have any life-threatening disease or other disease or condition which, according tothe investigator's judgment, could imply a potential hazard to the patient, orinterfere with the study participation or study outcome (e.g. chronic, unmanagedhepatitis infection)

14. be using aspirin, non-steroidal anti-inflammatory drugs (NSAIDS), herbs, naturalmedications, or other drugs with platelet inhibitory properties within one weekprior to surgery and for the duration of treatment with LR769

15. have active gastric or duodenal ulcer disease

16. have received a FVII- or FVIIa-containing product (either plasma derived orrecombinant) within 24 hours prior to administration of LR769

17. have a contraindication to antifibrinolytics

18. have planned combined major surgeries at the same time

19. be administered pharmacologic thromboprophylaxis within 5 half-lives of thatmedication before surgery or for the duration of treatment with LR769 -