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Haematology > Bleeding Disorders > Flashcards

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
Q

How to test mild and severe vWD?

A

Mild=dessmopressin

Severe = vwf concentrate (fVIII and vwf)

26
Q

Name 3 uncommon congenital bleeding disorders?

A

Factor XI deficiency, fibrinogen disorders, Factor XIII deficiency

27
Q

How can liver disease cause aquired bleeding disorders?

A

Liver produces clotting factors and thrombopoietin (platelet production)

28
Q

How does kidney disease lead to acquired bleeding disorders?

A

Increased urea affects platelet aggregation and also produces thrombopoietin

29
Q

What results would u expect in a case of DIC. 4 points

A

Decreased platelet count, increased APTT and pt, ddimers raised and fibrinogen decrease.

30
Q

Causes of DIC? Name 3

A

Infection with viruses or gram positive or negative bacteria

31
Q

What is immune thrombocytopenia purpura

A

Autoantibodies produced against platelets. Sensitised platelets removed by the spleen.

32
Q

If a patient

With immune thrombocytopenia purpura is resistant to steroid treatment what other options are there?

A

Splenectomy, if rheasus positive give anti d

33
Q

A deficiency of what protein causes thrombotic thrombocytopenia purpura? And what does that protein do?

A

Adamts13- responsible for cleavage of high MW multimers of vWf

34
Q

Name the thrombotic thrombocytopenic purpura Pentax of signs

A

1) microangiopathic haemolytic anaemia with schistocytes
2) severe thrombocytopenia
3) fever
4) renal impairment
5) neurological symptoms

35
Q

State Virchows triad

A

1) abnormal blood flow (stasis)
2) altered properties of the blood itself (hypercoagubility)
3) alterations in the endothelium (vessel wall damage)

36
Q

True or false - thrombosis can be venous and arterial

A

True

37
Q

What is the name given to inheritered or acquired disorders of haemostat in mechanisms that predispose to thrombosis?

A

Thrombophilia

38
Q

What causes arterial thrombosis? Clue=rupture

A

Rupture of artherosclerotic plaque

39
Q

What causes venous thrombosis?

A

Increase in systemic coagubility (endothelium usually remains intact)

40
Q

FV Leiden mutation is a risk factor for what?

A

Venous thrombosis

41
Q

What cleaves factor V?

A

Activated protein C (APC)

42
Q

What mutation causes the FV Leiden mutation found in 4% of Caucasians?

A

Arg 506 gln

43
Q

What is the prothrombin variant and what is its effect?

A

g20210a leads t increased prothrombin resulting in increased risk of thrombosis

44
Q

What is anti thrombin and how is its activity enhanced?

A

A protease that inhibits thrombin, IX, XA.0, XIA. Activity increased by binding to Heparin

45
Q

True or false type 1 AT deficiency is caused by qualitative defects of AT?

A

False

46
Q

What is protein C and how is it activated?

A

It is a serine protease activated by binding to thrombin.

47
Q

What’s does protein C inactivate?

A

FVa and FVIIIa

48
Q

What is protein S?

A

It is a cofactors of protein C

49
Q

What factors are Vit K dependent?

A

Ii, VII, IX, X, prC, prS

50
Q

What does warfarin inhibit?

A

Vit k epoxide reductase and Vit k reductase

51
Q

True or false- LMWHeparin has a longer half life than unfractionated heparin

A

True dat

52
Q

What is the mode of action of heparin?

A

Binds to antithrombin causing a conformational change

53
Q

True or false LMwH binds to thrombin but UFH does not

A

False

54
Q

True or false LMWH is cleared more slowly than UFH

A

True

55
Q

True or false- heparin decreases interaction of heparin bound antithrombin with fXa?

A

False- it increases interaction

Haematology (9 decks)

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