Haematology - Clotting Disorders + Tranfusion Reactions Flashcards

1
Q

what happens in DIC?

A

excessive activation of the coagulation pathways

microvascular thrombus forms leading to end organ failure

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2
Q

features of DIC?

A

bruising
purpura
bleeding
hypovolaemic shock

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3
Q

Ix for DIC and results?

A

FBC (low platelets)
PT (prolonged)
fibrinogen (low)
D dimers elevated

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4
Q

Tx for DIC?

A
treat cause
replace..
platelets
plasma
cryoprecipitate (fibrinogen)
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5
Q

what is a group and save?

what is a crossmatch?

A

G+S: lab processes patients blood type

crossmatch: lab actually provides blood

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6
Q

how is haemophilia inherited?

A

X linked

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7
Q

what types of haemophilia exist? what is deficient?

A

A - factor VIII (more common)

B - factor IX

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8
Q

features of haemophilia ?

A

haemaethroses
soft tissue bleed
prolonged bleeds

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9
Q

where is the problems in the clotting cascade in haemophilia?

A

platelet plug is able to form

secondary haemostasis cannot occur -> fibrin clot does not form

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10
Q

Ix for haemophilia?

A

PT (normal)

APTT (prolonged)

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11
Q

Tx for haemophilia?

A

factor VIII/IX concentrate

tranxemic acid

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12
Q

what happens in idiopathic thrombocytopenic purpura?

A

autoimmune condition where thrombocytopenia occurs

antibodies against platelets in circulation

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13
Q

features of idiopathic thrombocytopenic purpura?

A

preceding viral illness
petechia in LL/mucous membranes
bruising
mucosal bleeds

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14
Q

Tx for idiopathic thrombocytopenic purpura?

A

steroids
IV IG
platelets

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15
Q

Ix for idiopathic thrombocytopenic purpura? results?

A
FBC (low platelets)
blood film (low platelets)
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16
Q

who gets idiopathic thrombocytopenic purpura?

A

females
<10 (recover well)
>65

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17
Q

how is vWF deficiency inherted?

A

aut dom

18
Q

features of vWF deficiency?

A

mucosal bleeds
purpura
menorrhagia
bleeding haemorrhoids

19
Q

what does vWF normally do?

A

in primary haemostasis helps platelets stick together

carries factor VII

20
Q

Ix for vWF deficiency? result?

A
FBC (platelets can be down)
PT 
APTT (prolonged)
21
Q

Tx for vWF deficiency?

A

vWF concentrate (factor VIII)
platelets
cryoprecipitate
tranxemic acid for menorrhagia

22
Q

what happens in an immediate haemolytic transfusion reaction?

A

ABO mismatch

Ag-Ab complement activation occurs

23
Q

features of immediate haemolytic transfusion reaction?

A

IMMEDIATE
hypotension
fever
vasodilate

24
Q

Tx for immediate haemolytic transfusion reaction?

A

STOP
IV fluids
monitor BP/UO

25
Q

what happens in a delayed haemolytic transfusion reaction?

A

response to irregular Abs usually

5-10 days after transfusion

26
Q

how does delayed haemolytic transfusion reaction present?

A

5-10 days after
jaundice
renal failure

27
Q

Ix for delayed haemolytic transfusion reaction?

A

positive DAT
anaemic
high bilirubin/LDH

28
Q

what happens in a febrile non haemolytic transfusion reaction?

A

vasoactive release from the WBC in transfusions

often those getting repeated transfusions

29
Q

Tx for febrile non haemolytic transfusion reaction?

A

STOP
prove haemolysis not occuring
give anti pyretics (NSAID/paracetamol)

30
Q

what non haemolytic transfusion reactions can occur?

A

viral
bacterial
circulatory overload
urticarial

31
Q

if an urticarial reaction occurs, what do you do?

A

slow

antihistamines

32
Q

if a bacterial reaction occurs, what do you do?

A

prove not haemolytic reaction

Abs

33
Q

if circulatory overload occurs, what do you do?

A

diuretics

slow transfusion

34
Q

what are thrombophilias?

A

disorders of haemostasis that can predispose to thrombosis

35
Q

what are examples of familial thrombophilias?

A

factor V leiden
prothrombin 20210
antithrombin
protein C/S

36
Q

what are examples of acquired thrombophilias?

A

HIV

antiphospholipid syndrome

37
Q

Tx for thrombophilias?

A

short term prophylaxis

long term if recurrent VTE

38
Q

what does PT measure?

A

extrinsic pathway (fast blood clot)

TF:VIIa

39
Q

what does aPTT measure?

A

intrinsic pathway

VIIIa:IXa

40
Q

how to measure efficacy of LMWH?

A

anti Xa

41
Q

how to measure efficacy of unfractionated heparin?

A

APTT

42
Q

how does heparin work?

A

potentiates anti thrombin III