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CR2 > Haemostasis And Inherited Bleeding Disorders > Flashcards

Haemostasis And Inherited Bleeding Disorders Flashcards

1
Q

Primary haemostasis

A

Platelet adhesion and aggregation

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2
Q

Secondary haemostasis

A

Formation of fibrin network

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3
Q

Fibrinolysis

A

Clot breakdown

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4
Q

Lifespan of a platelet

A

8-14 days

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5
Q

What type of genetic material do platelets contain

A

RNA

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6
Q

What cells are platelets produced from and where

A

Megakaryocytes
Bone marrow

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7
Q

Contact with which molecule activates platelets

A

Endothelial collagen

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8
Q

What substances are released by activated platelets

A

Serotonin
TXA2
ADP
PL

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9
Q

What molecule does thrombin activate in the last step of the coagulation cascade

A

Fibrin

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10
Q

What turns the primary plug to a stable haemostatic plug

A

Fibrin

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11
Q

Which receptor on platelets does von willebrand factor bind to

A

Glycoprotein 1b receptor

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12
Q

What does von willebrand factor bind to

A

Collagen
Platelets

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13
Q

Which molecule connects platelets in platelet aggregation

A

Fibrin

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14
Q

What receptor does fibrin bind to on platelets in platelet aggregation

A

Glycoprotein iib/iiia receptor

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15
Q

Laboratory investigations for inherited bleeding and platelet disorders

A

Coagulation screen
Platelet count
Platelet function Test
Genetic tests

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16
Q

How are inherited bleeding and platelet disorders treated

A

Replacement of factors
Antifibrinolytics
DDAVP
FFP
platelets

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17
Q

Which types of condition affect primary haemostasis

A

Platelet defects
Collagen related disorders

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18
Q

What types of conditions affect secondary haemostasis

A

Factor deficiency
Anti phospholipid syndrome

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19
Q

Coagulation factor defects symptoms

A

Delayed onset of bleeding
Solitary large ecchymosis
Haemarthrosis

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20
Q

Platelet disorder/vWF symptoms

A

Spontaneous + immediate bleeding
Mucosal bleeding
Petechiae
Multiple small ecchymosis
Persistent bleeding at small cuts

21
Q

Ecchymosis

A

small bruise caused by blood leaking from broken blood vessels into the tissues of the skin or mucous membranes

22
Q

Where is the mutation that hpcauses haemophilia

A

X chromosome

23
Q

What causes haemophilia

A

Low level/ complete absence of coagulation factor VIII or IX

24
Q

Does haemophilia cause faster bleeding

A

No, just for longer period

25
Q

Does haemophilia affect primary or secondary haemostasis

A

Secondary

26
Q

What genetic abnormality causes mild haemophilia

A

Carrier of haemophilia gene

27
Q

What causes Haemophilia a

A

Deficiency of factor VIII

28
Q

What causes haemophilia b

A

Deficiency of factor IX

29
Q

What are the symptom differences between haemophilia a and b

A

None

30
Q

How is factor VIII/IX level affected by stress and pregnancy

A

Increases

31
Q

What is the normal level of factor VIII or IX

A

50-150 iu/dl

32
Q

Signs of haemophilia

A

Big lumpy bruises
Bleeding into joints
Spontaneous bleeding
Bleeding for long time
Excessive bleeding after childbirth - carriers
Serious internal bleeding into internal organs after insult

33
Q

What joints does bleeding most commonly occur into in haemophilia

A

Knees
Elbows
Ankles

34
Q

How can a bleed be recognised in haemophiliacs

A

Reduced movement
Pain
Heat swelling
Bruising - can travel

35
Q

What can cause deformation of joints in haemophiliacs

A

Constant long term bleeding into joints

36
Q

What medications are used to treat haemophilia

A

IV Recombinant or plasma factor VII/IX
SC DDAVP
IV/oral tranexamic acid

37
Q

How does DDAVP help in haemophilia

A

Makes platelets release contents including clotting factors

38
Q

How does tranexamic acid help haemophilia

A

Helps prevent blood clots breaking down

39
Q

How can gene therapy be used in haemophilia

A

Temporarily increase factor levels by releasing therapeutic gene code into liver cells

40
Q

Which chromosome has a mutation in von willebrands disease

A

Chromosome 12

41
Q

Are haemophilia and von willebrand disease sex dependent

A

Haemophilia - yes
Von willebrand - no

42
Q

Which is more common, von willebrands disease or haemophilia

A

Von willebrands disease

43
Q

Type 1 von willebrand disease

A

Reduction in von willebrand antigen and factor VIII as a result

44
Q

Type 2 vin willebrand disease

A

Vin willebrand factor doesn’t work properly

45
Q

Type 3 von willebrand factor

A

Extremely low levels/ complete absence of von willebrand antigen, RiCof, and factor VIII

46
Q

What does RiCof test

A

Von willebrand factor activity

47
Q

Vin willebrand disease symptoms

A

Nose bleeds
Easy bruising
Gum bleeds
Menorrhagia
Menstrual bleeding more than 7 days
Intestinal/gut bleeding

48
Q

Which medications are used for von willebrand disease treatment

A

IV Wilate and voncento
SC DDAVP
IV/ oral tranexamic acid

49
Q

What are wilate and voncento

A

FVIII and vWF replacement medications

CR2 (33 decks)

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