Case presentation

1. CASE PRESENTATION
ON
VON WILLEBRAND DISEASE TYPE-III
R. Anusha
Pharm D VI Year
Department of Clinical Pharmacy & Pharm D

2. INTRODUCTION
Von Willebrand Disease (VWD) is a genetic disorder caused by missing or defective Von
Willebrand Factor (VWF), a clotting protein.
VWF binds to Factor VIII, a key clotting protein and platelets in blood vessel walls,
which helps to form a platelet plug during the clotting process.
VWF is carried on chromosome 12 (short arm P) and occurs equally in men and women.
Sources of VWF:
VWF is derived from multiple sources of the body such as
1. Megakaryocytes
2. Endothelial cells

3. Sources of VWF
VWF is derived from multiple sources of the body such as
1. Megakaryocytes
2. Endothelial cells

4. Functions of VWF:
The two important function of VWF includes:
1. VWF binds and stabilises the factor VIII. In the presence of VWF, half-life of factor
VIII increases in circulation i.e, 12 hours. Without VWF, half-life of Factor VIII is 2.4
hours.
2. Mediates platelet adhesion to vascular injury site.
Without VWF, factor VIII cannot clot as well as the platelets in the blood vessels losing
their ability to form effectively.

5. TYPES OF VWD
TYPE Inheritance/Etiology Description % of VWD
affected patients
Congenital Type 1 Autosomal dominant Partial Quantitative
deficiency of VWF
About 75%
Type 2 Autosomal dominant Qualitative deficiency in
VWF
About 15-25%
Type 3 Autosomal recessive Complete absence of VWF Less than 5%
Acquired Autoimmune diseases Auto antibodies Rare

6. CLINICAL SYMPTOMS
• Epistaxis
• Easy bruising
• Post-operative bleeding
• Mucosal bleeding
• Menorrhagia
• Bleeding from the gums
• Excessive bleeding from a cut
or following a tooth extraction
or other dental procedure
• Blood in stool or urine

7. PATHOPHYSIOLOGY
• Structural or quantitative VWF abnormalities reduces platelet adhesion.
• Leads to muco-cutaneous hemorrhages of varying variety
• Severe quantitative VWF deficiency creates in addition factor VIII deficiency
• When factor VIII levels decrease to <30% of normal, anatomical soft tissue bleedings
also occurs.

8. DIAGNOSIS:
• Family history (bleeding history)
• Bleeding time
• aPTT (activated partial thromboplastin time)
• VWF antigen assay
• Ristocetin cofactor test
• Factor VIII activity
• Platelet aggregation test

9. TREATMENT
Treatment for VWD depends on the diagnosis and severity.
DDAVP (1-DEAMINO-8-D-ARGININE VASOPRESSIN):
It is synthetic version of a natural hormone vasopressin. It stimulates the release of VWF
from cells, which also increases factor VIII. It comes in 2 forms: injectables (0.3mcg/kg IV
or SC) and nasal spray (150-300 mcg).
Antifibrinolytics:
• Tranexamic acid may help in case of minor bleeding.
• Dose: 500mg oral.

10. Replacement therapy:
• Factor VIII conc. that contain VWF 40-60 U/Kg then 40-50 U/Kg q 12-24hr up to 7
days.
• Cryoprecipitate 1U/ 10 Kg OD.

11. CASE PRESENTATION

12. SUBJECTIVE FINDINGS
A female patient of 11 years old has been admitted in pediatric ward with chief
complaints of:
• Epistaxis
• Bleeding from scalp, due to head injury 4 days back.
K/C/O Von Willebrand Disease TYPE-III diagnosed at the age of 9 years.
FAMILY HISTORY:

13. 0BJECTIVE FINDINGS
LAB INVESTIGATIONS:
CBP :
• Hgb – 9.8gm%
• Platelets – 372000/cumm (seen in clumps)
• Bleeding time - >10min
• Activated partial thromboplastin time – 94.7sec
• Factor VIII assay – 3.3%
• VWF antigen assay - < 1%
GENERAL EXAMINATION:
Nose : epistaxis +
Head : bleeding +

14. ASSESSMENT
Based on subjective and objective findings the patient was diagnosed with
VON WILLEBRAND DISEASE TYPE-III

15. PLAN
S.NO DRUG DOSE FREQ ROA Day-1 Day-2 Day-3
1 T. Tranexamic acid 500 mg BD PO   
2 Von Willebrand
Factor
40 IU/Kg OD IV   
3 T. Acetaminophen 325mg BD PO   

16. PATIENT COUNSELLING
About disease:
Von Willebrand Disease (VWD) is a genetic disorder caused by missing or defective
Von Willebrand Factor (VWF), a clotting protein.
About Drugs:
T. Tranexamic acid:
This medication is used to treat heavy bleeding and it prevents prolonged bleeding.
Watch for active bleeding.

17. LIFE STYLE MODIFICATIONS:
• Avoid sports that could cause bruising, such as football, wrestling and hockey. Instead
they could stay active with activities like swimming and walking.
• Avoid aspirin and other NSAIDs for pain or fever. These drugs can increase the risk of
bleeding.
• Let your doctors know that you have Von Willebrand Disease before you have any type
of surgery.
• Tell your dentist about your history of heavy bleeding from minor injuries.
• Consume iron rich foods like green leafy vegetables, poultry, liver, grains and raisins.
• Exercise as part of maintaining healthy weight.

18. THANK YOU