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Haemostasis Flashcards

1
Q

what are the stages of primary haemostasis

A 
vasoconstriction (TXA2)
platelet adhesion
platelet activation
platelet secretion
platelet aggreggation - unstable plug
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2
Q

what causes platelet adhesion in primary haemostasis

A

disruption of the endothelium, exposing the sub endothelium –>
Collagens (GP1a/2a)
vWF (GP1b/F9/5)
factor VIII

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3
Q

what are the two types of granules secreted by platelets

A

dense:
amines (ADP and 5HT) -> aggregation of platelets

alpha:
factor V
vWF
fibrinogen
prothrombin
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4
Q

what stabilises platelet aggregation

A

fibrin

needs TXA2

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5
Q

what happens in platelet activation

A

platelets express active phospholipid and GP IIb and IIIa

causes production of TXA2

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6
Q

which factors are involved in the intrinsic pathway

A

XII
XI
IX
VIII

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7
Q

which factors are involved in the extrinsic pathway

A

VII

Tissue factor released from damaged subendothelium

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8
Q

which are the vitamin K dependant clotting factors

A 
II
VII
IX
X
protein s + c
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9
Q

what is the final product of the common pathway

A

fibrin

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10
Q

which clotting factor facilitates the cross linking of fibrin

A

XIII

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11
Q

what is the function of the extrinsic pathway

A

ignites secondary haemostasis

fast

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12
Q

what is the function of the intrinsic pathway

A

slower
involved in amplification
driven by thrombin

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13
Q

which factors in are activated by thrombin

A

XI
VIII
V
(V and VIII are cofactors)

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14
Q

what are clotting factors

A

serine proteases
made in liver
secreted as zymogens

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15
Q

what is measured by the PT

A

the extrinsic pathway

normal = 12

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16
Q

what is measured by the APTT

A

the intrinsic pathway

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17
Q

what is measured by the TT

A

the common pathway
normal = 18 seconds

tests the action of thrombin, presence/quality of fibrinogen

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18
Q

what is a secondary bleeding disorder

A

deficit in clotting factors

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19
Q

what is a primary bleeding disorder

A

a defect in platelets and blood vessels

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20
Q

which type of bleeding disorder is likely to produce prolonged bleeding after minor curs

A

primary as unable to clot with platelets

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21
Q

what is purpura indicative of

A

thrombocytopenia or vasculitis

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22
Q

petiche are present in which kind of bleeding disorder

A

primary

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23
Q

which type of bleeding disorder produces delayed severe bleeding post surgery

A

secondary

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24
Q

which type of bleeding disorders produce haemarthroses and muscle haematomas

A

secondary

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25
what is quantitate platelet disorders more commonly known as
``` thrombocytopenia reduced production: - BM failure - Wiskoff-Aldrich - congenital infection ``` increased destruction: - splenomegaly - intravascular thormbosis - IMMUNE
26
list some types of qualitative platelet disorders
inherited: - Glanzman's - Bernard Soulier acquired: - drugs - renal disease
27
what are the inherited blood vessel disorders which can cause primary bleeding disorders
- hereditary haemorrhagic telengiectasis | - connective tissue disease (Ehlor-Danlos, Marfan's, Pseudexanthoma elasticum)
28
what are the acquired blood vessel disorders which can cause primary bleeding disorders
- Henoch- Schonleu purpura - infection - drug reactions - prolonged steroid use - senile purpura - trauma - scurvy
29
what is the inheritance pattern for Hereditary hemorrhagic telengiectasia
autosomal dominant defect in TGF-beta signalling pathway needed for blood vessel development --> dilation of capillaries and small arterioles
30
what does hereditary haemorrhagic telengiectasia present with
small red blanching spots on skin and mucous membranes esp on nose and mouth
31
what is Henoch-Scholen purpura
``` allergic vasculitis (usually in children following an URTI) presents with: -arthropathy - GI symptoms - haematuria usually resolves spontaneously ```
32
what is Bernard- Soulier syndrome
lack of GPIb leading to a failure of platelet adhesion to vWF autosomal RECESSIVE
33
why can renal disease cause platelet disorders
uraemia inhibits platelet adhesion, aggregation and activation
34
what is Wiskott-Aldrich syndrome
``` X linked recessive presents with: -eczema, -thrombocytopenia, -immune deficiency platelets are too small and do not function properly ```
35
what happens in DIC
intravascular thrombosis from widespread generation of fibrin in BVs - ->consumption of platelets and clotting factors causes secondary activation of fibrinolysis - -> mixed initial thrombosis then increased bleeding time both secondary and primary mechanisms
36
what is TTP
microangiopathic thrombosis resulting in platelet consumption causing a profound thrombocytopenia
37
what is ITP
autoimmune thrombocytopenia acute in children chronic in adults presents as: easy bruising, purpura, epistaxis, menorrhagia
38
list some inherited coagulation (secondary haemostats) disorders
``` Von Willebrand Disease Haemophillia A (VIII deficiency) Haemophillia B (christmas disease IX deficiency) ``` raised APTT managed with factor replacement, desmopressin and transexamis acid
39
how is DIC diagnosed
increased bleeding time TT PT and APTT | decreased platelet and factor assays
40
what is the role of thrombin in the intrinsic pathway
accelerates it and catalysese the formation of XIIIa
41
what is the role of protein C in the common pathway and which disease is associated with it
inhibits activations of factor V factor 5 Leiden causes increased clotting mutation in structure of V so protein S cannot recognise it
42
how does von willibrands present
can be asymptomatic other than increased bleeding following surgery/dentist
43
how is plasmin formed from plasminogen
plasminogen produced in liver | catalysed by fibrin-tPA complex and urokinase
44
which test can be done to test for fibrin degradation products
D-dimer | catalysed by plasmin
45
what are the endogenous anti-coagulation agents
anti- thrombin - inhibits and thrombin protein c protein s c+s- inactivated V and VIII
46
what is the inheritance pattern of anti-thrombin deficiency
dominant rare can be resistant to heparin
47
what is antiphospholipid syndrome
antiphosphlipid antibodies can present with: thrombus, recurrent miscarriage will need anti-coagulating for life risks: VTE/PE miscarriage, still birth, pre-eclampsia, CVA, MI
48
list some vitamin K antagonists
warfarin acenocumerol phenindrone
49
list an exogenous antithrombin agent
dabigatran
50
list some anti factor Xa agents
rivaroxabran apixaban edoxaban
51
list 3 kinds of fibrinolytic drugs
recombinant tPA Streptokinase Urokinase
52
how does heparin work
increases the interaction between antithrombin and factor Xa
53
what is the antidote for heparin
protamine | alkaline protein which binds acidic heparin
54
describe a venous thrombi
red clots - fibrin and RBCs usually a result of stasis and hyper coagulability anticoag most effective treatment
55
desrcibe arterial thrombi
white clots - platelets and fibrin usually a result of atherosclerosis anti-platelets most effective treatment
56
what are the main side effects of heparin
osteoporosis HIT hyperkalaemia
57
what interacts with warfarin
``` ABX NSAIDS antiepileptics alcohol grapefruit cranberry ```
58
name 4 procoagulants
coag factors fresh frozen plasma cryoprecipitate vit K
59
what is warfarin's main action
inhibits vitamin K reductase
60
what is May and Turner's syndrome
compression of the left common iliac vein by the right common iliac artery therefore predisposing the individual to DVTs in the left leg
61
what is Paget-Schroetter disease
predisposition to forming DVTs in the arms owing to thoracic outlet syndrome (compression of the neuromuscular bundle at the superior thoracic outlet between the anterior and middle scalenes)

defence (12 decks)

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