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Related Subjects: |Sideroblastic Anaemia |Splenectomy |Blood Products - Platelets |Von Willebrand Disease
Patients with von Willebrand disease (vWD) often present with mucosal bleeding (e.g. epistaxis, menorrhagia, dental/surgical bleeding). โ ๏ธ Avoid aspirin & NSAIDs as they worsen bleeding. ๐ Tranexamic acid is frequently used to control episodes.
| Type | Inheritance | Clinical Features | Severity |
|---|---|---|---|
| Type 1 | AD | Mild mucosal bleeding (epistaxis, bruising, menorrhagia) | Mild (80% of cases) |
| Type 2 (A, B, M, N) | AD | Moderate bleeding, qualitative vWF defect | Moderate |
| Type 3 | AR | Severe: mucosal + joint bleeds (hemophilia-like) | Severe (rare) |
| Acquired | Secondary | Seen with autoimmune disease, malignancy, drugs | Variable |
๐ก Exam Tip: Both vWD and Hemophilia A show prolonged APTT, but vWD has mucosal bleeding & prolonged bleeding time, unlike Hemophilia.
| Test | Finding |
|---|---|
| Bleeding time | Prolonged |
| Platelet count | Normal |
| APTT | Prolonged (โ Factor VIII) |
| PT | Normal |
| Factor VIII & vWF levels | Low |
| Ristocetin assay | Impaired platelet aggregation (diagnostic) |
Teaching Point ๐ฉบ: vWD is the commonest inherited bleeding disorder. It causes mucocutaneous bleeding due to impaired platelet adhesion and, in severe cases, reduced factor VIII. Types: Type 1 (partial deficiency), Type 2 (dysfunctional vWF), Type 3 (severe deficiency). Management: tranexamic acid, desmopressin, or vWF concentrates depending on severity.