Bleeding disorders

Question 1

coagulation

Answer 1

a system of repair defects in vessel wall.

primary haemostasis= formation of platelet plug

secondary haemostasis= formation of fibrin clot

Question 2

normal haemostasis

Answer 2

procoagulant proteins
anticoagulant proteins
fibrinolytic system
vascular endothelium
platelets

Question 3

platelets

Answer 3

central role
initial closure in the vessel wall
platelet granules contain procoagulant and antioagulant
provide the membrane surface onto which the activated clotting factors binds

Question 4

coagulation

Answer 4

coagulation factors are plasma synthesised in liver. when activated lead in a sequence of reactions to the deposition of fibrin. originally thought of as a cascade of reactions but now viewed as a network of reactions integrally involved with platelets

Question 5

important coagulation factors/co-factors

Answer 5

factor VIII
factor IX
VWF

Question 6

causes of abnormal bleeding

Answer 6

vascular disorder
low platelets
platelet function defect
defective or deficient coagulation proteins

Question 7

assessment / accurate clinical history for bleeding

Answer 7

site of bleeding
duration of bleeding
precipitating cause
surgery/procedures
fix
systemic illness
drugs

Question 8

site of bleeding

Answer 8

bruising, purport, epistaxis, superficial cuts menorrhagia

• primary haemostat defect
• platelet abnormality or VWF

haemarthrosis and muscle bleeds

• secondary haemostat defect
• abnormal coagulation factor

recurrent bleeding from a single anatomical site
structural abnormality.

Question 9

systemic illness causing bleeding

Answer 9

renal disease
liver disease
sepsis
DIC
paraproteins
drugs

Question 10

drugs that cause bleeding

Answer 10

aspirin
antiplatelet drugs
NSAIDs
warfarin/heparin
chemotherapy

Question 11

investigations for bleeding

Answer 11

FBC
blood film exam
renal function
liver function
coagulation screen- APTT, PTT, fibrinogen
platelet function test (PFA-100)

Question 12

VWF

Answer 12

essential for platelet/subendothelial interaction and platelet interaction

PT normal
APTT often prolonged

Question 13

haemophilia A / B

Answer 13

haemoarthrosis
X linked recessive

mild, moderate, severe (depends on VIII levels)

severe often presents when become mobile

prolonged APPT

Question 14

immune thrombocytopenia

Answer 14

platelet count is 15
blood film
no platelet lumps
normal Hb and WBC

Question 15

myelodysplasia

Answer 15

low platelets
bone marrow dysfunction (ineffective haemopoiesis)
blood film diagnostic with abnormal platelets
quantitative + qualitative defect

Question 16

hereditary haemorrhage telangectasia

Answer 16

autosomal dominant
vascular malformation in skin, nasal membranes, gut, lungs, brain, liver
small feeding vessels which blanch on pressure

Question 17

DIC

Answer 17

Intravascular activation of coagulation
deposition of fibrin in microvasculature

causes- sepsis, malignancy, severe ill health

consumptive coagulopathy

low platelets
APPT and PTT prolonged
d dimer

treat with FFP and platelets

Question 18

senile purpura

Answer 18

atrophy of the supporting tissues of cutaneous blood vessels

worse on dorsal aspect of forarm and hand

worse if steroids