Get ready for the CAMLPR Hematology Test. Use our quizzes, flashcards, and detailed explanations to enhance your understanding. Prepare confidently for your exam!

Multiple Choice

The most common inherited bleeding disorder von Willebrand disease is often treated with which agent?

Desmopressin works by prompting the endothelium to release stored von Willebrand factor, along with a rise in factor VIII levels. This increases the ability of platelets to adhere to damaged vessels and forms a more stable clot, which is why it’s the most commonly effective initial treatment for mild-to-moderate von Willebrand disease. It is especially helpful in type 1 VWD, where there is a deficiency but the vWF that is present can function when released. It’s not effective in the most severe form (type 3), where little to no vWF is available, and in some subtypes like type 2B it can be problematic, requiring vWF-containing concentrates instead. Repeated use can lead to reduced response and there’s a risk of hyponatremia from fluid retention, so dosing and monitoring are important.

Desmopressin works by prompting the endothelium to release stored von Willebrand factor, along with a rise in factor VIII levels. This increases the ability of platelets to adhere to damaged vessels and forms a more stable clot, which is why it’s the most commonly effective initial treatment for mild-to-moderate von Willebrand disease. It is especially helpful in type 1 VWD, where there is a deficiency but the vWF that is present can function when released. It’s not effective in the most severe form (type 3), where little to no vWF is available, and in some subtypes like type 2B it can be problematic, requiring vWF-containing concentrates instead. Repeated use can lead to reduced response and there’s a risk of hyponatremia from fluid retention, so dosing and monitoring are important.