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Haemostasis And Inherited Bleeding Disorders Flashcards

(49 cards)

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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

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A

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

21
Q

Ecchymosis

Study These Flashcards
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

23
Q

What causes haemophilia

Study These Flashcards
A

Low level/ complete absence of coagulation factor VIII or IX

24
Q

Does haemophilia cause faster bleeding

Study These Flashcards
A

No, just for longer period

25
Does haemophilia affect primary or secondary haemostasis
Secondary
26
What genetic abnormality causes mild haemophilia
Carrier of haemophilia gene
27
What causes Haemophilia a
Deficiency of factor VIII
28
What causes haemophilia b
Deficiency of factor IX
29
What are the symptom differences between haemophilia a and b
None
30
How is factor VIII/IX level affected by stress and pregnancy
Increases
31
What is the normal level of factor VIII or IX
50-150 iu/dl
32
Signs of haemophilia
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
What joints does bleeding most commonly occur into in haemophilia
Knees Elbows Ankles
34
How can a bleed be recognised in haemophiliacs
Reduced movement Pain Heat swelling Bruising - can travel
35
What can cause deformation of joints in haemophiliacs
Constant long term bleeding into joints
36
What medications are used to treat haemophilia
IV Recombinant or plasma factor VII/IX SC DDAVP IV/oral tranexamic acid
37
How does DDAVP help in haemophilia
Makes platelets release contents including clotting factors
38
How does tranexamic acid help haemophilia
Helps prevent blood clots breaking down
39
How can gene therapy be used in haemophilia
Temporarily increase factor levels by releasing therapeutic gene code into liver cells
40
Which chromosome has a mutation in von willebrands disease
Chromosome 12
41
Are haemophilia and von willebrand disease sex dependent
Haemophilia - yes Von willebrand - no
42
Which is more common, von willebrands disease or haemophilia
Von willebrands disease
43
Type 1 von willebrand disease
Reduction in von willebrand antigen and factor VIII as a result
44
Type 2 vin willebrand disease
Vin willebrand factor doesn’t work properly
45
Type 3 von willebrand factor
Extremely low levels/ complete absence of von willebrand antigen, RiCof, and factor VIII
46
What does RiCof test
Von willebrand factor activity
47
Vin willebrand disease symptoms
Nose bleeds Easy bruising Gum bleeds Menorrhagia Menstrual bleeding more than 7 days Intestinal/gut bleeding
48
Which medications are used for von willebrand disease treatment
IV Wilate and voncento SC DDAVP IV/ oral tranexamic acid
49
What are wilate and voncento
FVIII and vWF replacement medications

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