Clotting factor disorders: The Daily PANCE Blueprint

The answer is B. Von Willebrand Disease (vWD)

Von Willebrand Disease is the most common inherited bleeding disorder and is characterized by a qualitative or quantitative deficiency of von Willebrand factor (vWF), which is essential for platelet adhesion. The clinical presentation often includes mucocutaneous bleeding, such as easy bruising and prolonged bleeding after procedures. The family history and normal platelet count with prolonged bleeding time are consistent with vWD.

Incorrect Answers:

A. Hemophilia A – This is an X-linked recessive disorder affecting males and characterized by factor VIII deficiency. It typically presents with hemarthrosis and deep tissue bleeding rather than mucocutaneous bleeding.

C. Idiopathic thrombocytopenic purpura (ITP) – ITP is characterized by autoimmune destruction of platelets, leading to a low platelet count, which is not seen in this patient.

D. Disseminated intravascular coagulation (DIC) – DIC is an acquired syndrome characterized by widespread activation of coagulation, leading to bleeding and thrombotic complications. It is typically associated with an underlying disease and presents with a combination of bleeding and thrombotic symptoms.

E. Vitamin K deficiency – This leads to deficiency of vitamin K-dependent clotting factors (II, VII, IX, X) and presents with bleeding. However, it does not typically present with a prolonged bleeding time and is not consistent with the patient’s family history.