Bleeding Disorders

Question 1

Deficiency: Factor VIII deficiency
Clinical Presentation: Deep tissue, joint, and post-surgical bleeding
Lab Findings:
PT: Normal
aPTT: Prolonged
Platelet Count: Normal

Answer 1

Hemophilia A

Question 2

Deficiency: Factor IX deficiency
Clinical Presentation: Deep tissue, joint, and post-surgical bleeding (similar to Hemophilia A)
Lab Findings:
PT: Normal
aPTT: Prolonged
Platelet Count: Normal

Answer 2

Hemophilia B

Question 3

Deficiency: vWF (defective or deficient), sometimes associated with low Factor VIII levels
Clinical Presentation: Mucosal bleeding (e.g., epistaxis, menorrhagia), prolonged bleeding from cuts
Lab Findings:
PT: Normal
aPTT: Normal or prolonged (if Factor VIII is affected)
Platelet Count: Normal

Answer 3

Von Willebrand Disease

Question 4

Deficiency: Consumptive coagulopathy (depletion of clotting factors and platelets)
Clinical Presentation: Diffuse bleeding (petechiae, ecchymoses, hematuria), organ ischemia/thrombosis
Lab Findings:
PT: Prolonged
aPTT: Prolonged
Platelet Count: Decreased

Answer 4

Disseminated Intravascular Coagulation (DIC)

Question 5

Deficiency: Platelet destruction due to autoantibodies
Clinical Presentation: Mucosal bleeding, petechiae, easy bruising
Lab Findings:
PT: Normal
aPTT: Normal
Platelet Count: Decreased

Answer 5

Immune Thrombocytopenic Purpura (ITP)

Question 6

Deficiency: ADAMTS13 deficiency (impaired vWF multimer cleavage)
Clinical Presentation: Pentad: Thrombocytopenia, hemolytic anemia, renal failure, neurologic symptoms, fever
Lab Findings:
PT: Normal
aPTT: Normal
Platelet Count: Decreased

Answer 6

Thrombotic Thrombocytopenic Purpura (TTP)

Question 7

Deficiency: GPIIb/IIIa deficiency (platelet aggregation defect)
Clinical Presentation: Mucosal bleeding, petechiae, menorrhagia
Lab Findings:
PT: Normal
aPTT: Normal
Platelet Count: Normal

Answer 7

Glanzmann Thrombasthenia

Question 8

Deficiency: GPIb deficiency (platelet adhesion defect)
Clinical Presentation: Mucosal bleeding, easy bruising, giant platelets on blood smear
Lab Findings:
PT: Normal
aPTT: Normal
Platelet Count: Decreased or normal

Answer 8

Bernard-Soulier Syndrome

Question 9

Deficiency: Decreased synthesis of Factors II, VII, IX, X, Protein C, and S
Clinical Presentation: Bleeding, especially post-surgical or mucosal
Lab Findings:
PT: Prolonged
aPTT: Prolonged
Platelet Count: Normal

Answer 9

Vitamin K Deficiency

Question 10

Deficiency/Mutation: Mutation in Factor V making it resistant to inactivation by activated Protein C (APC resistance)
Clinical Presentation: Hypercoagulability; increased risk of deep vein thrombosis (DVT) and pulmonary embolism (PE), rarely arterial clots
Lab Findings:
PT: Normal
aPTT: Normal (may show resistance to prolongation with activated Protein C assay)
Platelet Count: Normal

Answer 10

Factor V Leiden

Question 11

Deficiency/Mechanism: Platelet dysfunction due to toxins in renal failure, which impair platelet adhesion and aggregation
Clinical Presentation: Mucosal bleeding, petechiae, prolonged bleeding time
Lab Findings:
PT: Normal
aPTT: Normal
Platelet Count: Normal

Answer 11

Uremic Platelet Dysfunction

Question 12

Clotting Factors Acted On: Inhibits synthesis of Vitamin K-dependent clotting factors: II, VII, IX, X, and anticoagulant proteins C and S
Important Fact: Requires regular INR monitoring due to variable dosing and drug interactions; antidote is Vitamin K

Answer 12

Warfarin

Question 13

Clotting Factors Acted On: Activates antithrombin, which inhibits Factors IIa (thrombin) and Xa
Important Fact: Monitored via aPTT; antidote is protamine sulfate

Answer 13

Heparin

Question 14

Clotting Factors Acted On: Primarily inhibits Factor Xa with some inhibition of IIa (thrombin)
Important Fact: Longer half-life than unfractionated heparin; no routine monitoring required

Answer 14

Enoxaparin (Low Molecular Weight Heparin)

Question 15

Clotting Factors Acted On: Primarily inhibits Factor Xa with some inhibition of IIa (thrombin)
Important Fact: Similar to enoxaparin; fewer bleeding complications compared to unfractionated heparin

Answer 15

Dalteparin (Low Molecular Weight Heparin)

Question 16

Clotting Factors Acted On: Selectively inhibits Factor Xa by binding to antithrombin
Important Fact: No antidote; low risk of heparin-induced thrombocytopenia (HIT)

Answer 16

Fondaparinux (Low Molecular Weight Heparin)

Question 17

Clotting Factors Acted On: Directly inhibits Factor IIa (thrombin)
Important Fact: Used in patients with HIT; no antidote

Answer 17

Bivalirudin

Question 18

Clotting Factors Acted On: Direct thrombin (Factor IIa) inhibitor
Important Fact: Derived from hirudin (leech saliva); used in HIT; no antidote

Answer 18

Lepirudin

Question 19

Clotting Factors Acted On: Direct thrombin (Factor IIa) inhibitor
Important Fact: Used in HIT and for patients undergoing percutaneous coronary intervention (PCI); metabolized by liver

Answer 19

Argatroban

Question 20

Clotting Factors Acted On: Direct thrombin (Factor IIa) inhibitor
Important Fact: Used for prophylaxis of venous thromboembolism (VTE) after hip surgery; no antidote

Answer 20

Desirudin

Question 21

Clotting Factors Acted On: Direct thrombin (Factor IIa) inhibitor
Important Fact: Oral anticoagulant; antidote is idarucizumab

Answer 21

Dabigatran

Question 22

Clotting Factors Acted On: Direct Factor Xa inhibitor
Important Fact: Oral anticoagulant; used for stroke prevention in atrial fibrillation; no routine monitoring required

Answer 22

Apixaban

Question 23

Clotting Factors Acted On: Direct Factor Xa inhibitor
Important Fact: Oral anticoagulant; requires less renal function monitoring compared to rivaroxaban

Answer 23

Edoxaban

Question 24

Clotting Factors Acted On: Direct Factor Xa inhibitor
Important Fact: Oral anticoagulant; used for prevention of DVT, PE, and stroke in atrial fibrillation; antidote is andexanet alfa

Answer 24

Rivaroxaban

Question 25

Cofactors dependent of Vitamin K:______________

Answer 25

2, 7, 9 10