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Cardiorespiratory > Haemostasis - Bleeding: Disorders of Coagulation > Flashcards

Haemostasis - Bleeding: Disorders of Coagulation Flashcards

1
Q

what is occurring in secondary haemostasis?

A

stabilisation of the platelet plug with fibrin

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2
Q

what is the role of the coagulation?

A

to generate thrombin IIa which will convert fibrinogen to fibrin

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3
Q

deficiency of any coagulation factor results in?

A

failure of thrombin generation and hence fibrin formation

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4
Q

list 3 causes of coagulation factor deficiencies

A

deficiency of coagulation factor production
dilution
increased consumption

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5
Q

list a hereditary cause for deficiency of coagulation factor

A

haemophilia A/B > factor VIII/IX

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6
Q

list an acquired cause for deficiency of coagulation factor

A 
liver disease (decreased production)
anticoagulant drugs (warfarin, DOACs)
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7
Q

list an acquired cause for dilution of coagulation factors

A

blood transfusion

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8
Q

list an acquired cause of increased consumption

A

disseminated intravascular coagulation (DIC)

immune - autoantibodies

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9
Q

haemophilia A causes a deficiency in what coagulation factor?

A

factor VIII

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10
Q

haemophilia B causes a deficiency in what coagulation factor?

A

factor IX

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11
Q

what is haemarthrosis?

A

bleeding into the joints (hallmark of haemophilia)

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12
Q

chronic haemarthrosis can lead to?

A

permanent damage > limit movement > muscle wasting

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13
Q

what medical procedure should be avoided in patients with haemophilia?

A

intramuscular injections

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14
Q

how does deficiency if factors VIII and IX present?

A

haemophilia: severe but compatible with life, spontaneous joint and muscle bleeding

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15
Q

how does deficiency if factor II (prothrombin) present?

A

lethal

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16
Q

how does deficiency of factor XI present?

A

bleed after trauma but not spontaneously

17
Q

how does deficiency of factor XII present?

A

no bleeding at all

18
Q

how does transfusion lead to dilution?

A

red cell transfusions no longer contain plasma

19
Q

what is disseminated intravascular coagulation?

A

condition where blood clots are excessively formed in the body’s blood vessels

20
Q

disseminated intravascular coagulation is associated with?

A

sepsis
major tissue damage
inflammation

21
Q

clinical features of coagulation disorders

A

superficial cuts do not bleed, bruising is common, nosebleeds are rare, spontaneous bleeding is deep, into muscles and joints, bleeding after trauma may be delayed and is prolonged, bleeding frequently restarts after stopping

22
Q

clinical distinction between bleeding due to platelet and coagulation defects

A

platelet/vascular: superficial bleeding into skin, mucosal membranes, bleeding immediate after injury
coagulation: bleeding into deep tissues, muscles, joints, delayed, but severe bleeding after injury, often prolonged

23
Q

tests for coagulation disorders

A

screening (clotting screen): prothrombin time, activated partial thromboplastin time, FBC
coagulation factor assays
tests for inhibitors

24
Q

the activated partial prothrombin time measures what pathway?

A

intrinsic

25
Q

the prothrombin time measures what pathway?

A

extrinsic

26
Q

what could cause normal PT but high APPT?

A

haemophilia A + B

factor XI, XII deficiencies

27
Q

what could cause high PT but normal APPT?

A

factor VII deficiency

28
Q

what could cause high PT and high APPT?

A

Liver disease
Anticoagulant drugs e.g. warfarin
DIC (platelets and D dimer)
Dilution following red cell transfusion

29
Q

principles of treatment of abnormal haemostasis (failure of production/function)

A

replace missing factors/platelets (prophylactic, therapeutic), stop drugs e.g. aspirin, NSAIDs

30
Q

principles of treatment of abnormal haemostasis (immune destruction)

A

Immunosuppression (e.g. prednisolone)

Splenectomy for ITP

31
Q

principles of treatment of abnormal haemostasis (increased consumption)

A

Treat cause

Replace as necessary

32
Q

factor replacement therapy

A

plasma (fresh frozen plasma)
cryoprecipitate
factor concentrates
recombinant forms of FVII and FIX

33
Q

fresh frozen plasma contains?

A

Contains all coagulation factors

34
Q

cryoprecipitate contains?

A

Rich in Fibrinogen, FVIII, VWF, Factor XIII

35
Q

factor concentrates contain?

A

Concentrates available for all factors except factor V.

Prothrombin complex concentrates (PCCs) Factors II, VII, IX, X

36
Q

novel treatments for haemophilia

A

gene therapy
bispecific antibodies
RNA silencing

37
Q

how do bispecific antibodies work as a treatment for haemophilia?

A

Binds to FIXa and FX

Mimics procoagulant function of FVIII

38
Q

how does RNA silencing work as a treatment for haemophilia?

A

target natural anticoagulant - antithrombin

39
Q

list additional haemostatic treatments

A

desmopressin (increase VWF, vasopressin analogue)
tranexamic acid (antifibrinolytic)
fibrin spray/glue
hormonal (OCP for menorrhagia)

Cardiorespiratory (20 decks)

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