Hemostasis: Blood Coagulation and Fibrinolysis - Part 4

Question 1

Which bleeding disorders are due to inherited disorder of the clotting pathway?

Answer 1

Hemophilia A and B

Question 2

Which bleeding disorders are due to defects in platelet plug formation?

Answer 2

-Von Willebrand disease
-Platelet defects:
- Thrombocytopenia
- Bernard Soulier syndrome
- Thrombasthenia of Glanzman and
Naegeli

Question 3

In hemophilia, there, which is an x-linked inherited coagulation disorder, there is a defect in what?

Answer 3

Intrinsic coagulation pathway (increased clotting time and increased APTT)

Question 4

Patients with hemophilia A have a deficiency in which coagulation factor?

Answer 4

Factor VIII

Question 5

Patients with hemophilia B have a deficiency in which coagulation factor?

Answer 5

Factor IX

Question 6

What are characteristics of patients with Hemophilia A and B?

Answer 6

• Tendency towards easy bruising
• Massive hemorrhage after trauma and surgical procedures
• Spontaneous hemorrhages, particularly in the joints -hemarthrosis

Question 7

What is hemarthrosis?

Answer 7

• Spontaneous hemorrhages, particularly in the joints

- characteristic of Hemophilia

Question 8

How is the inheritance in Von Willebrand Disease?

Answer 8

Either dominant or recessive

Question 9

What are the clinical features of a patient with Von Willebrand Disease?

Answer 9

Presents with the clinical features similar to hemophiliaA

• Increased mucosal bleeding
• Epistaxis
• Increased post-operative bleeding

Question 10

What are the lab findings in a patient with Von Willebrand Disease?

Answer 10

Bleeding time –Prolonged (indicates a defect in platelet plug formation)

Platelet count –Normal

APTT –Prolonged (Factor VIII levels may be low normal)

PT (INR) –Normal (Extrinsic pathway is normal)

vWFlevels -LOW

Question 11

What happens to bleeding time due to platelet plug formation?

Answer 11

Increased

Question 12

What is the difference between a quantitative defect, such as thrombocytopenia, and qualitative defects, such as Bernard-Soulier or Thrombasthenia of Glansman and Naegeli?

Answer 12

Quantitative defects:

  - Low platelet count
  - Increased bleeding time

Qualitative defects:

   - Normal platelet count
   - Increased bleeding time

Question 13

What is the deficiency in Bernard-Soulier Syndrome?

Answer 13

GpIb

Question 14

What is the deficiency in Glanzmann thrombasthenia?

Answer 14

GpIIb-IIIa