A 20-year-old woman with a family history of von Willebrand disease is found to have an activated paial thromboplastin time (aPTT) of 78 (normal = 32) on routine testing prior to cholecystectomy. Fuher investigation reveals a prothrombin time (PT) of 13 (normal = 12), a platelet count of 350,000/mm3 , and an abnormal bleeding time. Which of the following should be administered in the perioperative period?
A 20-year-old woman with a family history of von Willebrand disease is found to have an activated paial thromboplastin time (aPTT) of 78 (normal = 32) on routine testing prior to cholecystectomy. Fuher investigation reveals a prothrombin time (PT) of 13 (normal = 12), a platelet count of 350,000/mm3 , and an abnormal bleeding time. Which of the following should be administered in the perioperative period?
💡 Explanation
**Core Concept**
The patient's prolonged activated partial thromboplastin time (aPTT) indicates a coagulation pathway defect, specifically involving the intrinsic or common coagulation pathway. Von Willebrand disease is a genetic disorder affecting the von Willebrand factor (VWF), a crucial protein involved in platelet adhesion and coagulation factor VIII stabilization.
**Why the Correct Answer is Right**
The patient's abnormal bleeding time, coupled with a normal prothrombin time (PT), suggests a platelet-related issue, which is further confirmed by the elevated platelet count. This points towards a qualitative platelet defect, as seen in von Willebrand disease. Desmopressin (DDAVP) is a synthetic analogue of vasopressin, which stimulates the release of VWF from storage sites in endothelial cells, thereby correcting the bleeding time in patients with type 1 von Willebrand disease.
**Why Each Wrong Option is Incorrect**
**Option A:** Fresh frozen plasma (FFP) would not be effective in correcting the bleeding time, as it lacks a significant amount of VWF. FFP is more suitable for correcting PT and aPTT abnormalities due to coagulation factor deficiencies.
**Option B:** Platelet transfusion would not address the underlying VWF deficiency, and it may even lead to an adverse reaction in patients with von Willebrand disease.
**Option C:** Cryoprecipitate contains high levels of VWF, but its use is not indicated in type 1 von Willebrand disease, as it can cause thrombotic complications.
**Clinical Pearl / High-Yield Fact**
Desmopressin is a first-line treatment for mild to moderate von Willebrand disease, as it stimulates the release of VWF from storage sites in endothelial cells, thereby correcting the bleeding time.
**Correct Answer:** D. Desmopressin (DDAVP)
✓ Correct Answer: D. Desmopressin (DDAVP)
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