Week 6

Question 1

What are vascular bleeding disorders?

Answer 1

Conditions characterized by easy bruising and spontaneous bleeding from small vessels due to abnormalities in blood vessels or connective tissues.

Question 2

What are the symptoms of vascular bleeding disorders?

Answer 2

Petechiae (small red blood spots), ecchymoses (small bruises), and purpuras (large bruises).

Question 3

Name an inherited vascular bleeding disorder.

Answer 3

Hereditary Haemorrhagic Telangiectasia (HHT).

Question 4

What causes Hereditary Haemorrhagic Telangiectasia (HHT)?

Answer 4

Mutations in ENG, ALK1, or BMP9 genes affecting VEGF production and angiogenesis.

Question 5

What is Senile Purpura?

Answer 5

A condition in older adults caused by the loss of skin elasticity and vascular collagen atrophy, leading to easy bruising.

Question 6

How does scurvy cause bleeding disorders?

Answer 6

Vitamin C deficiency leads to defective collagen synthesis, weakening blood vessels and causing bleeding.

Question 7

Thrombocytopenia

Answer 7

Low Platelet Count

Question 8

What are common causes of thrombocytopenia?

Answer 8

Bone marrow failure, leukemia, chemotherapy, drug toxicity, and viral infections.

Question 9

How is thrombocytopenia treated?

Answer 9

platelet transfusions, and in some cases, corticosteroids or immunosuppressants.

Question 10

What is the primary cause of ITP?

Answer 10

Autoantibodies targeting platelet glycoproteins (e.g., GPIIb/IIIa), leading to platelet destruction.

Question 11

How is ITP treated?

Answer 11

Corticosteroids, IV immunoglobulins, splenectomy

Question 12

What enzyme deficiency causes TTP?

Answer 12

ADAMTS13 deficiency, leading to uncontrolled von Willebrand Factor (VWF) multimers and excessive clotting.

Question 13

What are the key features of TTP?

Answer 13

Thrombocytopenia, microangiopathic hemolytic anemia,

Question 14

treatment for TTP

Answer 14

Plasma exchange (plasmapheresis) to remove autoantibodies

Question 15

What is Bernard-Soulier Syndrome?

Answer 15

A rare platelet disorder caused by defective GPIb, preventing platelet adhesion to VWF.

Question 16

What is Glanzmann’s Thrombasthenia?

Answer 16

A rare disorder caused by defective GPIIb/IIIa, preventing platelet aggregation.

Question 17

How are platelet function disorders diagnosed?

Answer 17

Using platelet aggregometry, flow cytometry, and blood smears.

Question 18

What is the difference between Haemophilia A and B?

Answer 18

Haemophilia A is a deficiency in Factor VIII, while Haemophilia B is a deficiency in Factor IX.

Question 19

Why is haemophilia more common in males?

Answer 19

It is an X-linked recessive disorder, meaning males with the defective gene express the disease.

Question 20

How is haemophilia treated?

Answer 20

Factor replacement therapy, gene therapy, and prophylactic treatment with Emicizumab.

Question 21

What is the role of Desmopressin (DDAVP) in haemophilia?

Answer 21

It stimulates the release of stored Factor VIII from endothelial cells, used in mild haemophilia A.

Question 22

What is the function of von Willebrand Factor (VWF)?

Answer 22

It mediates platelet adhesion to damaged vessels and protects Factor VIII from degradation.

Question 23

How is VWD treated?

Answer 23

Desmopressin (DDAVP) for mild cases, or VWF-containing factor concentrates for severe cases.

Question 24

What clotting factors depend on Vitamin K?

Answer 24

Factors II, VII, IX, and X, as well as Proteins C and S.

Question 25

What is the standard prevention for VKDB in newborns?

Answer 25

Intramuscular Vitamin K injection at birth.

Question 26

What is DIC?

Answer 26

Disseminated Intravascular Coagulation A condition where excessive clotting depletes clotting factors, leading to both thrombosis and bleeding.

Question 27

What are common causes of DIC?

Answer 27

Sepsis, trauma, malignancy, obstetric complications, and severe infections.

Question 28

Why does Factor V Leiden cause thrombosis?

Answer 28

It resists degradation by activated Protein C, leading to prolonged clotting activity.

Question 29

How is Antithrombin Deficiency diagnosed?

Answer 29

Using Antithrombin activity assays and genetic testing.

Question 30

How does Heparin prevent clotting?

Answer 30

It enhances Antithrombin’s inhibition of thrombin and Factor Xa.

Question 31

How does Warfarin work?

Answer 31

It inhibits Vitamin K recycling, reducing activation of clotting factors II, VII, IX, and X.

Question 32

How does Aspirin prevent thrombosis?

Answer 32

inhibits COX-1, reducing thromboxane A2 and preventing platelet aggregation.

Question 33

How can infection cause vascular bleeding disorders?

Answer 33

Bacterial and viral infections (e.g., measles) can damage blood vessels, leading to purpura.

Question 34

How can viral infections trigger ITP?

Answer 34

Viral antigens may bind to platelets, causing the immune system to mistakenly attack them

Question 35

What is the role of ADAMTS13 in normal haemostasis?

Answer 35

It cleaves large von Willebrand Factor multimers to prevent excessive platelet aggregation.

Question 36

What happens in acquired TTP?

Answer 36

Autoantibodies inhibit ADAMTS13, leading to excessive clot formation.

Question 37

How does Caplacizumab treat TTP?

Answer 37

It is an antibody that prevents VWF from binding to platelets, reducing clot formation.

Question 38

What is Storage Pool Deficiency?

Answer 38

A platelet disorder where granules fail to release contents needed for clotting, leading to excessive bleeding.

Question 39

How is platelet function tested?

Answer 39

Using Light Transmission Aggregometry (LTA) and Flow Cytometry to detect platelet surface glycoproteins.

Question 40

What is VWF:Ag?

Answer 40

A lab test that measures von Willebrand Factor antigen levels in the blood.

Question 41

What is Ristocetin-Induced Platelet Agglutination (RIPA)?

Answer 41

A test to diagnose Type 2B VWD, where a low concentration of Ristocetin causes abnormal platelet aggregation.

Question 42

What is VWF Multimer Analysis?

Answer 42

A test that uses gel electrophoresis to assess the size and structure of VWF multimers.

Question 43

How does COVID-19 impact coagulation?

Answer 43

It can cause endothelial dysfunction, increased thrombin generation, and thrombotic microangiopathy, increasing DIC risk.