Mind maps Bleeding disorders

Question 1

Examples

Answer 1

Bernard-soulier syndrome
Haemophilia
Vitamin K insufficiency
Glanzmann's thrombasthenia
Von Willebrand Disease

Question 2

What is Bernard-soulier syndrome

Answer 2

Autosomal recessive bleeding disorder

Deficiency of glycoprotein Ib

Question 3

Cause of Bernard-soulier syndrome

Answer 3

Hereditary condition that leads to deficiency of glycoprotein Ib

Question 4

Investigations of Bernard-soulier syndrome

Answer 4

Increased bleeding time

Normal or low platelet count

Question 5

Conservative treatment of Bernard-soulier syndrome

Answer 5

Patient education

Question 6

Medical treatment of Bernard-soulier syndrome

Answer 6

Desmospressin may decrease bleeding time

Recombinant activated factor VII

Question 7

**What are the 4 steps of clot formation

Answer 7

Vessel constriction
Platelet adhesion and aggregation
Blood coagulation
Fibrinolysis

Question 8

What is Haemophilia

Answer 8

Inherited condition that impairs the body’s ability to coagulate the blood

Question 9

Types of Haemophilia

Answer 9

Hereditary condition with 2 forms:
Type A = lack of factor VIII
Type B = lack of factor IX

Question 10

Investigations of Haemophilia

Answer 10

Normal prothrombin time

Increased partial thromboplastin time

Question 11

Conservative treatment of Haemophilia

Answer 11

Patient education

Avoid aspirin, NSAIDs, heparin and warfarin

Question 12

Medical treatment of Haemophilia

Answer 12

Replace deficient clotting factor with regular infusions

Question 13

Diseases that affect the Platelet adhesion and aggregation step of clot formation

Answer 13

Glanzmann’s thrombasthenia
von Willebrand disease
Bernard-Soulier syndrome

Question 14

Diseases that affect the blood coagulation step of clot formation

Answer 14

Haemophilia

Question 15

Diseases that affect the fibrinolysis step of clot formation

Answer 15

Antiplasmin deficiency

Question 16

What is vitamin K insufficiency

Answer 16

Avitaminosis occurring when there is decreased vitamin K1 or K2 or both. This results in:

• decreased synthesis of factors 2, 7, 9, 10
• decreased synthesis of proteins C and S

Question 17

Causes of vitamin K insufficiency

Answer 17

Drugs e.g. warfarin
Malnutrition
Malabsorption
Alcoholism
Cystic fibrosis
CKD
Cholestatic disease

Question 18

Investigations of vitamin K insufficiency

Answer 18

Increased prothombin time

Normal or increased partial thromboplastin time

Question 19

Conservative treatment of vitamin K insufficiecny

Answer 19

Patient education

Dietary advice about food rich in vitamin K

Question 20

Medical treatment of vitamin K insufficiency

Answer 20

Treat cause

Vitamin K supplements

Question 21

What is Glanzmann’s thrombasthenia

Answer 21

This is a rare autosomal recessive or acquired autoimmune condition in which platelets are deficient of glycoprotein IIb/IIIa
GpIIb/IIIa binds fibrinogen

Question 22

What is function of glycoprotein IIb/IIIa

Answer 22

binds fibrinogen

Question 23

Causes of Glanzmann’s thrombasthenia

Answer 23

Disease of hereditary or acquired autoimmune cause

Question 24

Investigations of Glanzmann’s thrombasthenia

Answer 24

Increased bleeding time

Question 25

Conservative treatment of Glanzmann's thrombasthenia

Answer 25

Patient education | Avoid aspirin and NSAIDs

Question 26

Medical treatment of Glanzmann's thrombasthenia

Answer 26

Desmopressin | Recombinant activated factor VII

Question 27

What is Von Willebrand disease

Answer 27

Hereditary coagulation disorder, which involves a defect in von Willebrand factor (VWF)

Question 28

What is the most common hereditary coagulation disorder

Answer 28

Von Willebrand disease

Question 29

What is the function of Von Willebrand factor (VWF)

Answer 29

Bind glycoprotein Ib receptor on platelets to subendothelial collagen

Question 30

Causes/types of Von Willebrand disease

Answer 30

Hereditary condition Many types, but most common: Type 1, Type 2, Type 3 and Type Normandy

Question 31

Investigations of Von Willebrand disease

Answer 31

``` Increased activated partial thromboplastin time Increased bleeding time Normal prothrombin time Decreased VWF antigen Decreased factor VIIIc ```

Question 32

Conservative treatment of Von Willebrand disease

Answer 32

Patient education | Avoid aspirin and NSAIDs

Question 33

Medical treatment of Von Willebrand disease

Answer 33

Desmospressin may be useful but is not helpful in type 3 Von Willebrand disease