Coagulation & bleeding disorders

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USMLE Step 2 CK › Coagulation & bleeding disorders

Questions 1 - 5
1

Which of the following is the most appropriate agent to reverse his anticoagulation?

Vitamin K

Protamine sulfate

Idarucizumab

Andexanet alfa

Explanation

Apixaban is a direct oral anticoagulant that acts as a Factor Xa inhibitor. In cases of life-threatening bleeding, specific reversal is indicated. Andexanet alfa is a recombinant modified factor Xa protein that acts as a decoy, binding to and sequestering Factor Xa inhibitors, thereby restoring the activity of endogenous Factor Xa. Protamine sulfate reverses heparin. Vitamin K reverses warfarin. Idarucizumab is the specific reversal agent for dabigatran, a direct thrombin inhibitor.

2

Which of the following is the most appropriate first-line therapy to improve hemostasis in this patient?

Vitamin K

Desmopressin (DDAVP)

Platelet transfusion

Fresh frozen plasma

Explanation

Patients with chronic kidney disease and uremia develop a qualitative platelet defect (uremic platelet dysfunction), characterized by impaired platelet adhesion and aggregation. This occurs despite a typically normal platelet count and coagulation panel. The most appropriate first-line pharmacologic agent for treating or preventing bleeding in this setting is desmopressin (DDAVP), which increases the release of factor VIII and von Willebrand factor from endothelial cells, improving platelet function. Dialysis is also a key intervention to reduce uremic toxins. Platelet transfusions are generally ineffective as the transfused platelets quickly become dysfunctional in the uremic environment.

3

Which of the following blood products will most effectively increase his fibrinogen level?

Fresh frozen plasma

Platelets

Cryoprecipitate

Packed red blood cells

Explanation

The patient has a complex coagulopathy from liver failure, including a critically low fibrinogen level (hypofibrinogenemia), which contributes significantly to his bleeding. Cryoprecipitate is the blood product that contains the most concentrated source of fibrinogen. It also contains factor VIII, factor XIII, and von Willebrand factor. While fresh frozen plasma (FFP) contains all clotting factors including fibrinogen, it is not as concentrated, and large volumes would be required to significantly raise the fibrinogen level. Packed red blood cells and platelets do not contain significant amounts of fibrinogen.

4

Which of the following is the most appropriate immediate treatment?

Infusion of cryoprecipitate

Infusion of recombinant factor IX concentrate

Administration of desmopressin (DDAVP)

Administration of fresh frozen plasma

Explanation

This patient has hemophilia B, which is a deficiency of factor IX. He is presenting with an acute hemarthrosis, a common and serious complication. The cornerstone of management is prompt replacement of the deficient clotting factor. Therefore, infusion of recombinant factor IX concentrate is the most appropriate and specific treatment. Cryoprecipitate is rich in factor VIII, vWF, and fibrinogen, but not factor IX. Desmopressin is used for mild hemophilia A and vWD but is not effective for hemophilia B. FFP contains factor IX but is not concentrated, requiring large volumes, and is not the standard of care when specific factor concentrates are available.

5

This infant's condition is most likely due to a deficiency of which of the following?

Platelets

Factor VIII

Fibrinogen

Vitamin K

Explanation

This clinical scenario is classic for vitamin K deficiency bleeding (VKDB) of the newborn, formerly known as hemorrhagic disease of the newborn. Risk factors include home birth, lack of routine neonatal care (no vitamin K prophylaxis), and exclusive breastfeeding (breast milk is low in vitamin K). Vitamin K is essential for the synthesis of factors II, VII, IX, and X. The deficiency leads to a prolonged PT (due to factor VII's short half-life) and eventually a prolonged aPTT. Treatment is administration of vitamin K.