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Haematology > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

what is haemophilia

A

an X linked hereditary disorder in which abnormally prolonged bleeding recurs episodically at oe or a few sites on each occasion

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2
Q

what is Haemophilia A

A

factor VIII deficiency

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3
Q

what is haemophilia B

A

factor IX deficiency

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4
Q

describe the features of haemophilia

A

no abnormality of primary haemostasis
bleeding from medium to large blood vessels
mild moderate and severely affected families depending on factor VIII/IX level

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5
Q

what does prothrombin time screen for

A

Tissue factor/VIIa

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6
Q

what does APTT screen for

A

VIII/IXa

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7
Q

what does APTT stand for

A

activate partial thromboplastin time

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8
Q

what are the clinical features of severe haemophilia

A

recurrent haemarthroses
recurrent soft tissue bleeds-bruising in toddlers
prolonged bleeding after dental extractions, surgery and invasive procedures

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9
Q

what does heparin do

A

prevents activation factors 2,9,10,11

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10
Q

warfarin

A

affects synthesis of factors 2,7,9,10

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11
Q

describe blood test abnormalities in haemophilia

A

APTT prolonged PT normal

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12
Q

what is thrombophilia

A

familial or acquired disorders of the haemostatic mechanism which are likely to predispose to thrombosis

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13
Q

potential mechanisms of thrombophilia

A

increase in coagulation activity
decrease in fibrinolytic activity
decrease in anticoagulant activity

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14
Q

name some of the naturally occurring anticoagulants

A

Serine protease inhibitors

Protein C and Protein S

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15
Q

name some hereditary thrombophilia

A 
Factor V Leiden
Prothrombin 20210 mutation 
Antithrombin deficiency 
Protein C deficiency 
Protein S deficiency
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16
Q

if you have a hereditary thrombophilia how do you treat

A

depending on the clinical picture-only offer anticoagulation if RECURRENT thrombotic events

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17
Q

why in hereditary throbophilias do you not always anticoagulate

A

many factors influence the patients risk of thrombosis beyond the genetic predisposition

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18
Q

name an acquired thrombophilia

A

antiphospholipid antibody syndrome

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19
Q

what carries a greater risk factor for thrombosis acquired or hereditary thrombophilia

A

acquired thrombophilia

20
Q

what are the features of antiphospholipid syndrome

A

recurrent thrombosis-arterial including TIA and venous
recurrent fetal loss
mild thrombocytopenia

21
Q

in terms of haemostasis what does antiphospholipid syndrome cause

A

conformational change in B2 glycoprotein which leads to activation of both primary and secondary haemostasis and vessel wall abnromalities

22
Q

how is antiphospholipid syndrome treated

A

aspirin and warfarin

23
Q

what does heparin do

A

potentiates antithrombin with immediate effect

24
Q

what are the 2 forms of heparin

A

unfractioned and LMWH

25
Q

how do you monitor heprain

A

for unfractioned APTT

for LMWH-anti Xa assay but usually no monitoring of LMWH is required as more predictable response

26
Q

what are the complications of heparin

A

bleeding

heparin induced thrombocytopenia (wit thrombosis) HITT

27
Q

because of the risk of HITT what do you monitor patients on heparin for

A

FBC

28
Q

what is a long term complication of heparin

A

osteoporosis

29
Q

describe the soluble properties of vit K

A

fat soluble vitamin

30
Q

where is vitamin K absorbed

A

upper intestine

31
Q

what does vitamin K require for absorbption

A

bile salts

32
Q

what does vitamin K do

A

final carboxylation of clotting factors II, VII, IX and X

33
Q

how should warfarin be taken

A

dose should be taken at same time every day

34
Q

how is the INR calculated

A

patients PT in seconds divided by mean normal PT in seconds

35
Q

give an example of a Xa inhibitor

A

Rivaroxaban

36
Q

give an example of thrombin inhibitors

A

dabigatran

37
Q

what is the most common hereditary blood-clotting disorder

A

won Willebrand disease

38
Q

what is von Willebrand factor required for

A

platelet adhesion

39
Q

how is von Willebrand disease most commonly inherited

A

autosomal dominant

40
Q

what does von Willebrand usually present with

A

varying degrees of bleeding tendency usually from bruising, nosebleeds and bleeding gums

41
Q

describe the blood results of someone with von Willebrand disease

A

usually get elevated PT. APTT may be prolonged, factor VIII decreased (vwF is bound to Factor VIII)

42
Q

what role does von Willebrand factor have in blood clotting

A

endothelial (vessel wall) damage exposes collagen and releases von Willebrand factor and other proteins to which platelets have receptors-platelet adhesion at site of injury

43
Q

what is the treatment of von Willebrand disease

A

transexamic acid for mild bleeding, desmopressin can also increase levels of vWF by inducing the release of vWF from endothelial cells

44
Q

what type of disease is Factor V Leiden

A

most common inherited thrombophilia

45
Q

how is Facor V Leiden inherited

A

either homozygous or heterozygous

46
Q

after VTE do you screen for Factor V Leiden

A

isn’t recommended as previous thromboembolism itself is risk factor for further events and this should dictate specific management in the future rather than the thrombophilia identified

Haematology (14 decks)

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