Efficacy and Safety of Fanhdi®, a High-purity Von Willebrand Containing FVIII Concentrate, in Pediatric Patients With Von Willebrand Disease

Inclusion Criteria:

1. Subjects diagnosed with severe (type 2 or 3) hereditary VWD (VWF:RCo<15-20 IU/dL),or VWF:Act<15-20 IU/dL.

2. Subjects under 6 years of age.

3. Signed informed consent form (ICF) provided by an authorized representative onbehalf of the subject in accordance with local law and institutional policy.

Exclusion Criteria:

1. Subjects diagnosed with acquired VWD.

2. Subjects with active bleeding at the time of the first infusion or within 10 daysprior to the infusion.

3. Subjects who have been treated with DDAVP or another FVIII containing VWFconcentrate during the 5 days prior to the infusion of the Fanhdi. Thistreatment-free period may be reduced to 3 days for subjects with type 3 VWD.

4. Subject who are positive for anti-VWF or anti-FVIII antibodies (≥0.5 Bethesda Units)or has been positive in the history of their disease.

5. Subjects with a known allergies/intolerance to any substance contained in Fanhdi.

6. Subjects with a known history of anaphylactic reaction(s) to blood or bloodcomponents.

7. Subjects presenting severe platelet activity dysfunction due to the use of drugs (aspirin, other nonsteroidal anti-inflammatory drugs [NSAIDs], etc.) or a congenitalor acquired platelet function disorder or other concomitant processes that mayinterfere with coagulation.

8. Subjects have a known previous infection with hepatitis A virus (HAV), hepatitis Bvirus (HBV), hepatitis C virus (HCV), or human immunodeficiency virus (HIV), or haveclinical signs and symptoms consistent with current HAV, HBV, HCV or HIV infection.

9. Subjects presenting anemia (hemoglobin <11 g/dL).

10. Subjects diagnosed with metabolic diseases that are not clinically controlled, suchas diabetes mellitus, which could potentially interfere with the interpretations ofthe study.

11. Participated in another clinical trial within 30 days prior to the screening visitor has received any investigational product (IP) within 3 months prior to thescreening visit.

12. If it is anticipated that the subject will be treated with other products containingFVIII or VWF different from Fanhdi throughout the subject's participation.

13. Subjects who, in the opinion of the investigator, may have compliance problems withthe protocol.