Mind maps Bleeding disorders Flashcards

1
Q

Examples

A
Bernard-soulier syndrome
Haemophilia
Vitamin K insufficiency
Glanzmann's thrombasthenia
Von Willebrand Disease
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2
Q

What is Bernard-soulier syndrome

A

Autosomal recessive bleeding disorder

Deficiency of glycoprotein Ib

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3
Q

Cause of Bernard-soulier syndrome

A

Hereditary condition that leads to deficiency of glycoprotein Ib

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4
Q

Investigations of Bernard-soulier syndrome

A

Increased bleeding time

Normal or low platelet count

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5
Q

Conservative treatment of Bernard-soulier syndrome

A

Patient education

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6
Q

Medical treatment of Bernard-soulier syndrome

A

Desmospressin may decrease bleeding time

Recombinant activated factor VII

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7
Q

**What are the 4 steps of clot formation

A

Vessel constriction
Platelet adhesion and aggregation
Blood coagulation
Fibrinolysis

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8
Q

What is Haemophilia

A

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

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9
Q

Types of Haemophilia

A

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

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10
Q

Investigations of Haemophilia

A

Normal prothrombin time

Increased partial thromboplastin time

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11
Q

Conservative treatment of Haemophilia

A

Patient education

Avoid aspirin, NSAIDs, heparin and warfarin

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12
Q

Medical treatment of Haemophilia

A

Replace deficient clotting factor with regular infusions

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13
Q

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

A

Glanzmann’s thrombasthenia
von Willebrand disease
Bernard-Soulier syndrome

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14
Q

Diseases that affect the blood coagulation step of clot formation

A

Haemophilia

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15
Q

Diseases that affect the fibrinolysis step of clot formation

A

Antiplasmin deficiency

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16
Q

What is vitamin K insufficiency

A

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
17
Q

Causes of vitamin K insufficiency

A
Drugs e.g. warfarin
Malnutrition
Malabsorption
Alcoholism
Cystic fibrosis
CKD
Cholestatic disease
18
Q

Investigations of vitamin K insufficiency

A

Increased prothombin time

Normal or increased partial thromboplastin time

19
Q

Conservative treatment of vitamin K insufficiecny

A

Patient education

Dietary advice about food rich in vitamin K

20
Q

Medical treatment of vitamin K insufficiency

A

Treat cause

Vitamin K supplements

21
Q

What is Glanzmann’s thrombasthenia

A

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

22
Q

What is function of glycoprotein IIb/IIIa

A

binds fibrinogen

23
Q

Causes of Glanzmann’s thrombasthenia

A

Disease of hereditary or acquired autoimmune cause

24
Q

Investigations of Glanzmann’s thrombasthenia

A

Increased bleeding time

25
Q

Conservative treatment of Glanzmann’s thrombasthenia

A

Patient education

Avoid aspirin and NSAIDs

26
Q

Medical treatment of Glanzmann’s thrombasthenia

A

Desmopressin

Recombinant activated factor VII

27
Q

What is Von Willebrand disease

A

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

28
Q

What is the most common hereditary coagulation disorder

A

Von Willebrand disease

29
Q

What is the function of Von Willebrand factor (VWF)

A

Bind glycoprotein Ib receptor on platelets to subendothelial collagen

30
Q

Causes/types of Von Willebrand disease

A

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

31
Q

Investigations of Von Willebrand disease

A
Increased activated partial thromboplastin time
Increased bleeding time
Normal prothrombin time
Decreased VWF antigen
Decreased factor VIIIc
32
Q

Conservative treatment of Von Willebrand disease

A

Patient education

Avoid aspirin and NSAIDs

33
Q

Medical treatment of Von Willebrand disease

A

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