Hemostasis: Blood Coagulation and Fibrinolysis - Part 4 Flashcards Preview

Medical Biochemistry > Hemostasis: Blood Coagulation and Fibrinolysis - Part 4 > Flashcards

Flashcards in Hemostasis: Blood Coagulation and Fibrinolysis - Part 4 Deck (14):
1

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

Hemophilia A and B

2

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

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

3

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

Intrinsic coagulation pathway (increased clotting time and increased APTT)

4

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

Factor VIII

5

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

Factor IX

6

What are characteristics of patients with Hemophilia A and B?

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

7

What is hemarthrosis?

-Spontaneous hemorrhages, particularly in the joints
-characteristic of Hemophilia

8

How is the inheritance in Von Willebrand Disease?

Either dominant or recessive

9

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

Presents with the clinical features similar to hemophiliaA
-Increased mucosal bleeding
-Epistaxis
-Increased post-operative bleeding

10

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

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

11

What happens to bleeding time due to platelet plug formation?

Increased

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?

Quantitative defects:
- Low platelet count
- Increased bleeding time

Qualitative defects:
- Normal platelet count
- Increased bleeding time

13

What is the deficiency in Bernard-Soulier Syndrome?

GpIb

14

What is the deficiency in Glanzmann thrombasthenia?

GpIIb-IIIa