Safety and Efficacy of 3 Different Doses of Long Acting Factor VII in Haemophilia A or B Patients With Inhibitors

Inclusion Criteria:

- Male haemophilia A or B patients with inhibitors

- Willing to undergo a bleeding preventive regimen of 3 months' duration and a total
trial length of approximately 8 months

- Historical or ongoing high titre inhibitor (more than or equal to 5 BU) based on
either medical records, laboratory report reviews, patient and/or care provider
interviews

- At least 2 bleeding episodes requiring bypassing haemostatic-drug-based treatment
within the last month or 12 bleeding episodes within the last 6 months prior to
observation period

- Body weight between 30 and 100 kg (both inclusive)

Exclusion Criteria:

- Body Mass Index (BMI) above 30 kg/m2

- Immune tolerance induction therapy within the last month prior to entering observation
phase period

- Known active pseudo tumours

- Platelet count less than 50,000 platelets/microL (based on local laboratory value at
screening visit)

- Congenital or acquired coagulation disorders other than haemophilia A or B

- Surgery within one month prior to the observation period. Catheter, stents and dental
extractions do not count as surgeries, i.e. they will not exclude the patient. Port
insertion is classified as surgery

- Scheduled major and/or orthopaedic surgery, during the trial period until Follow up
visit. Catheter, stents and dental extractions do not count as surgeries and will not
exclude the patient. Port insertion is classified as surgery

- Advanced atherosclerotic disease (i.e. known history of ischemic heart disease, or
ischemic stroke)

- Any clinical signs or known history of thromboembolic events incl. known deep vein
thrombosis (DVT)

- Known or clinically suspected allergy to activated recombinant human factor VII
(NovoSeven®/NovoSeven RT®/Niastase®)

- Prothrombin Time (PT) prolongation (30% above normal limits, or more than 5 seconds
compared to control or International Normalised Range (INR) more than 1.7 as defined
by local laboratory ranges at screening visit

- Severe liver disease (ALAT more than 4 times of the upper limit of normal reference
range) (as defined by local laboratory ranges) within a year of enrolment or at the
screening

- Clinical signs of renal dysfunction (dialysis) and/or creatinine levels more than or
equal to 20% above upper normal limit (according to local laboratory range at the
screening visit)

- Dosing of any investigational drug within the last 30 days prior to the present trial

- Any disease or condition which, according to the investigator's judgement, could imply
a potential hazard to the subject, interfere with the trial participation or trial
outcome

- HIV positive patients who either have low CD4+ lymphocyte count ( 200/microL or less
based on medical records within 6 months or laboratory screening at screening visit),
or who are HCV-PCR positive (based on medical records), or who both have low CD4+
lymphocyte count (200/microL or less) and are HCV-PCR positive. If HCV-PCR testing is
not locally available, a HIV positive patient who is HCV antibody positive cannot be
included

- Need to use other PEGylated pharmaceutical drug during the trial period