The coagulation profile in a 13-year old girl with menorrhagia having von Willebrand’s disease is ?
## **Core Concept**
Von Willebrand's disease (VWD) is a common inherited bleeding disorder caused by deficiency or dysfunction of **von Willebrand factor (VWF)**, a protein crucial for platelet adhesion and stabilization of **factor VIII**. This condition primarily affects females, especially those with menorrhagia.
## **Why the Correct Answer is Right**
The correct answer shows a **prolonged bleeding time**, **normal to decreased platelet count**, **normal PT (Prothrombin Time)**, and **prolonged aPTT (activated partial thromboplastin time)**. This profile is characteristic of VWD because:
- **Bleeding time** is prolonged due to impaired platelet adhesion.
- **aPTT** is prolonged because VWF stabilizes **factor VIII**, and its deficiency leads to quicker degradation of factor VIII, affecting the intrinsic coagulation pathway.
- **PT** remains normal as the extrinsic pathway, primarily involving factors VII, X, V, II (prothrombin), and fibrinogen, is not directly affected by VWF.
## **Why Each Wrong Option is Incorrect**
- **Option A:** Incorrect because it shows a normal bleeding time, which contradicts the expected impairment in platelet adhesion seen in VWD.
- **Option B:** Incorrect as it indicates a prolonged PT, which is not characteristic of VWD.
- **Option D:** Incorrect because it shows a decreased platelet count (thrombocytopenia), which is not a feature of VWD; the platelet count is usually normal.
## **Clinical Pearl / High-Yield Fact**
A key point to remember is that VWD is the most common inherited bleeding disorder and often presents with **menorrhagia** in females. Diagnosis involves measuring **VWF antigen**, **VWF activity**, and **factor VIII levels**. Desmopressin (DDAVP) is a common treatment to increase VWF and factor VIII levels.
## **Correct Answer:** .