Haematology - Clotting Disorders + Tranfusion Reactions Flashcards
what happens in DIC?
excessive activation of the coagulation pathways
microvascular thrombus forms leading to end organ failure
features of DIC?
bruising
purpura
bleeding
hypovolaemic shock
Ix for DIC and results?
FBC (low platelets)
PT (prolonged)
fibrinogen (low)
D dimers elevated
Tx for DIC?
treat cause replace.. platelets plasma cryoprecipitate (fibrinogen)
what is a group and save?
what is a crossmatch?
G+S: lab processes patients blood type
crossmatch: lab actually provides blood
how is haemophilia inherited?
X linked
what types of haemophilia exist? what is deficient?
A - factor VIII (more common)
B - factor IX
features of haemophilia ?
haemaethroses
soft tissue bleed
prolonged bleeds
where is the problems in the clotting cascade in haemophilia?
platelet plug is able to form
secondary haemostasis cannot occur -> fibrin clot does not form
Ix for haemophilia?
PT (normal)
APTT (prolonged)
Tx for haemophilia?
factor VIII/IX concentrate
tranxemic acid
what happens in idiopathic thrombocytopenic purpura?
autoimmune condition where thrombocytopenia occurs
antibodies against platelets in circulation
features of idiopathic thrombocytopenic purpura?
preceding viral illness
petechia in LL/mucous membranes
bruising
mucosal bleeds
Tx for idiopathic thrombocytopenic purpura?
steroids
IV IG
platelets
Ix for idiopathic thrombocytopenic purpura? results?
FBC (low platelets) blood film (low platelets)
who gets idiopathic thrombocytopenic purpura?
females
<10 (recover well)
>65
how is vWF deficiency inherted?
aut dom
features of vWF deficiency?
mucosal bleeds
purpura
menorrhagia
bleeding haemorrhoids
what does vWF normally do?
in primary haemostasis helps platelets stick together
carries factor VII
Ix for vWF deficiency? result?
FBC (platelets can be down) PT APTT (prolonged)
Tx for vWF deficiency?
vWF concentrate (factor VIII)
platelets
cryoprecipitate
tranxemic acid for menorrhagia
what happens in an immediate haemolytic transfusion reaction?
ABO mismatch
Ag-Ab complement activation occurs
features of immediate haemolytic transfusion reaction?
IMMEDIATE
hypotension
fever
vasodilate
Tx for immediate haemolytic transfusion reaction?
STOP
IV fluids
monitor BP/UO
what happens in a delayed haemolytic transfusion reaction?
response to irregular Abs usually
5-10 days after transfusion
how does delayed haemolytic transfusion reaction present?
5-10 days after
jaundice
renal failure
Ix for delayed haemolytic transfusion reaction?
positive DAT
anaemic
high bilirubin/LDH
what happens in a febrile non haemolytic transfusion reaction?
vasoactive release from the WBC in transfusions
often those getting repeated transfusions
Tx for febrile non haemolytic transfusion reaction?
STOP
prove haemolysis not occuring
give anti pyretics (NSAID/paracetamol)
what non haemolytic transfusion reactions can occur?
viral
bacterial
circulatory overload
urticarial
if an urticarial reaction occurs, what do you do?
slow
antihistamines
if a bacterial reaction occurs, what do you do?
prove not haemolytic reaction
Abs
if circulatory overload occurs, what do you do?
diuretics
slow transfusion
what are thrombophilias?
disorders of haemostasis that can predispose to thrombosis
what are examples of familial thrombophilias?
factor V leiden
prothrombin 20210
antithrombin
protein C/S
what are examples of acquired thrombophilias?
HIV
antiphospholipid syndrome
Tx for thrombophilias?
short term prophylaxis
long term if recurrent VTE
what does PT measure?
extrinsic pathway (fast blood clot)
TF:VIIa
what does aPTT measure?
intrinsic pathway
VIIIa:IXa
how to measure efficacy of LMWH?
anti Xa
how to measure efficacy of unfractionated heparin?
APTT
how does heparin work?
potentiates anti thrombin III
