Haematology - Clotting Disorders + Tranfusion Reactions

Question 1

what happens in DIC?

Answer 1

excessive activation of the coagulation pathways

microvascular thrombus forms leading to end organ failure

Question 2

features of DIC?

Answer 2

bruising
purpura
bleeding
hypovolaemic shock

Question 3

Ix for DIC and results?

Answer 3

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

Question 4

Tx for DIC?

Answer 4

treat cause
replace..
platelets
plasma
cryoprecipitate (fibrinogen)

Question 5

what is a group and save?

what is a crossmatch?

Answer 5

G+S: lab processes patients blood type

crossmatch: lab actually provides blood

Question 6

how is haemophilia inherited?

Answer 6

X linked

Question 7

what types of haemophilia exist? what is deficient?

Answer 7

A - factor VIII (more common)

B - factor IX

Question 8

features of haemophilia ?

Answer 8

haemaethroses
soft tissue bleed
prolonged bleeds

Question 9

where is the problems in the clotting cascade in haemophilia?

Answer 9

platelet plug is able to form

secondary haemostasis cannot occur -> fibrin clot does not form

Question 10

Ix for haemophilia?

Answer 10

PT (normal)

APTT (prolonged)

Question 11

Tx for haemophilia?

Answer 11

factor VIII/IX concentrate

tranxemic acid

Question 12

what happens in idiopathic thrombocytopenic purpura?

Answer 12

autoimmune condition where thrombocytopenia occurs

antibodies against platelets in circulation

Question 13

features of idiopathic thrombocytopenic purpura?

Answer 13

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

Question 14

Tx for idiopathic thrombocytopenic purpura?

Answer 14

steroids
IV IG
platelets

Question 15

Ix for idiopathic thrombocytopenic purpura? results?

Answer 15

FBC (low platelets)
blood film (low platelets)

Question 16

who gets idiopathic thrombocytopenic purpura?

Answer 16

females
<10 (recover well)
>65

Question 17

how is vWF deficiency inherted?

Answer 17

aut dom

Question 18

features of vWF deficiency?

Answer 18

mucosal bleeds
purpura
menorrhagia
bleeding haemorrhoids

Question 19

what does vWF normally do?

Answer 19

in primary haemostasis helps platelets stick together

carries factor VII

Question 20

Ix for vWF deficiency? result?

Answer 20

FBC (platelets can be down)
PT 
APTT (prolonged)

Question 21

Tx for vWF deficiency?

Answer 21

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

Question 22

what happens in an immediate haemolytic transfusion reaction?

Answer 22

ABO mismatch

Ag-Ab complement activation occurs

Question 23

features of immediate haemolytic transfusion reaction?

Answer 23

IMMEDIATE
hypotension
fever
vasodilate

Question 24

Tx for immediate haemolytic transfusion reaction?

Answer 24

STOP
IV fluids
monitor BP/UO

Question 25

what happens in a delayed haemolytic transfusion reaction?

Answer 25

response to irregular Abs usually | 5-10 days after transfusion

Question 26

how does delayed haemolytic transfusion reaction present?

Answer 26

5-10 days after jaundice renal failure

Question 27

Ix for delayed haemolytic transfusion reaction?

Answer 27

positive DAT anaemic high bilirubin/LDH

Question 28

what happens in a febrile non haemolytic transfusion reaction?

Answer 28

vasoactive release from the WBC in transfusions | often those getting repeated transfusions

Question 29

Tx for febrile non haemolytic transfusion reaction?

Answer 29

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

Question 30

what non haemolytic transfusion reactions can occur?

Answer 30

viral bacterial circulatory overload urticarial

Question 31

if an urticarial reaction occurs, what do you do?

Answer 31

slow | antihistamines

Question 32

if a bacterial reaction occurs, what do you do?

Answer 32

prove not haemolytic reaction | Abs

Question 33

if circulatory overload occurs, what do you do?

Answer 33

diuretics | slow transfusion

Question 34

what are thrombophilias?

Answer 34

disorders of haemostasis that can predispose to thrombosis

Question 35

what are examples of familial thrombophilias?

Answer 35

factor V leiden prothrombin 20210 antithrombin protein C/S

Question 36

what are examples of acquired thrombophilias?

Answer 36

HIV | antiphospholipid syndrome

Question 37

Tx for thrombophilias?

Answer 37

short term prophylaxis | long term if recurrent VTE

Question 38

what does PT measure?

Answer 38

extrinsic pathway (fast blood clot) TF:VIIa

Question 39

what does aPTT measure?

Answer 39

intrinsic pathway VIIIa:IXa

Question 40

how to measure efficacy of LMWH?

Answer 40

anti Xa

Question 41

how to measure efficacy of unfractionated heparin?

Answer 41

APTT

Question 42

how does heparin work?

Answer 42

potentiates anti thrombin III