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Bleeding Disorders Flashcards

1
Q

What causes essential thrombocytopenia?

A

Overproduction of platelets by megakaryocytes in BM. Myloproliferative disorder

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2
Q

Is essential thrombocytopenia bar-abl positive?

A

No

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3
Q

What is the median age of ET

A

50

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4
Q

What is erythrometagia and what bleeding disorder is it associated with?

A

ET, burning hands and feet

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5
Q

Transitory visual or hearing defects are associated with what bleeding disorder

A

ET

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6
Q

Risk of thrombosis is increased in patients with ET if it is accompanied by two features. What are they?

A

Jak2 mutation (V615F in 50%)and high wbc

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7
Q

All patients with ET are given what?

A

Aspirin

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8
Q

10% of patients with et have what mutation?

A

MPL

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9
Q

Et can be treated with what drugs?

A

Oral hydroxycarbamide or anagrelied or interferon

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10
Q

What’s are the most common factor deficiencies

A

Haemophilia a and b and von willibrand

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11
Q

What is haemophilia A a deficiency of and is it more common in men or women

A

Factor VIII, Men (sex linked)

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12
Q

What is the range for sever haemophilia A?

A
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13
Q

What s the occurrence of haemophilia A per million

A

30-100

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14
Q

Flip tip rearrangement is common in which gene?

A

fVIII gene

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15
Q

Progressive joint deformity and disability found in what bleeding disorder?

A

Haemophilia A

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16
Q

What treatment is given to mild haemophilia A and severe haemophilia A?

A

dessmopressin is mild and prophylactic regular factor if severe

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17
Q

What factor deficiency causes Haemophilia B

A

Factor IX

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18
Q

True or false: a 50:50 mix in a coagulation screen will correct haemophilia B

A

True

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19
Q

What is von willibrand factor?

A

A carrier protein for FVIII. Acts as a bridge between platelets and subendothelial collagen fibres

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20
Q

What two abnormalities cause vwf disease?

A

Decrease in plasma concentration of vwf or decreased activity of structurally abnormal vWF

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21
Q

How is vwf synthesised?

A

As a polypeptide- forms multimers with high molecular weight

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22
Q

True or false APTT will be normal is an individual with vWD?

A

True

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23
Q

Difference between type 1,2 and 3 vWD

A

Type 1= partial quantitative deficiency
Type 2= qualitative deficiency
Type 3= complete deficient of vwf

24
Q

What is used to treat mild vWD?

A

Tranexamic acid (an antifibrinolytic prevents fibrinolysis)

25
How to test mild and severe vWD?
Mild=dessmopressin | Severe = vwf concentrate (fVIII and vwf)
26
Name 3 uncommon congenital bleeding disorders?
Factor XI deficiency, fibrinogen disorders, Factor XIII deficiency
27
How can liver disease cause aquired bleeding disorders?
Liver produces clotting factors and thrombopoietin (platelet production)
28
How does kidney disease lead to acquired bleeding disorders?
Increased urea affects platelet aggregation and also produces thrombopoietin
29
What results would u expect in a case of DIC. 4 points
Decreased platelet count, increased APTT and pt, ddimers raised and fibrinogen decrease.
30
Causes of DIC? Name 3
Infection with viruses or gram positive or negative bacteria
31
What is immune thrombocytopenia purpura
Autoantibodies produced against platelets. Sensitised platelets removed by the spleen.
32
If a patient | With immune thrombocytopenia purpura is resistant to steroid treatment what other options are there?
Splenectomy, if rheasus positive give anti d
33
A deficiency of what protein causes thrombotic thrombocytopenia purpura? And what does that protein do?
Adamts13- responsible for cleavage of high MW multimers of vWf
34
Name the thrombotic thrombocytopenic purpura Pentax of signs
1) microangiopathic haemolytic anaemia with schistocytes 2) severe thrombocytopenia 3) fever 4) renal impairment 5) neurological symptoms
35
State Virchows triad
1) abnormal blood flow (stasis) 2) altered properties of the blood itself (hypercoagubility) 3) alterations in the endothelium (vessel wall damage)
36
True or false - thrombosis can be venous and arterial
True
37
What is the name given to inheritered or acquired disorders of haemostat in mechanisms that predispose to thrombosis?
Thrombophilia
38
What causes arterial thrombosis? Clue=rupture
Rupture of artherosclerotic plaque
39
What causes venous thrombosis?
Increase in systemic coagubility (endothelium usually remains intact)
40
FV Leiden mutation is a risk factor for what?
Venous thrombosis
41
What cleaves factor V?
Activated protein C (APC)
42
What mutation causes the FV Leiden mutation found in 4% of Caucasians?
Arg 506 gln
43
What is the prothrombin variant and what is its effect?
g20210a leads t increased prothrombin resulting in increased risk of thrombosis
44
What is anti thrombin and how is its activity enhanced?
A protease that inhibits thrombin, IX, XA.0, XIA. Activity increased by binding to Heparin
45
True or false type 1 AT deficiency is caused by qualitative defects of AT?
False
46
What is protein C and how is it activated?
It is a serine protease activated by binding to thrombin.
47
What's does protein C inactivate?
FVa and FVIIIa
48
What is protein S?
It is a cofactors of protein C
49
What factors are Vit K dependent?
Ii, VII, IX, X, prC, prS
50
What does warfarin inhibit?
Vit k epoxide reductase and Vit k reductase
51
True or false- LMWHeparin has a longer half life than unfractionated heparin
True dat
52
What is the mode of action of heparin?
Binds to antithrombin causing a conformational change
53
True or false LMwH binds to thrombin but UFH does not
False
54
True or false LMWH is cleared more slowly than UFH
True
55
True or false- heparin decreases interaction of heparin bound antithrombin with fXa?
False- it increases interaction

Haematology (9 decks)

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