bleeding disorders

Question 1

normal haemostatic mechanisms

Answer 1

vessel wall
platelets
von willebrand factor
coagulation factors

Question 2

primary response

Answer 2

platelet plug

vWF

Question 3

secondary response

Answer 3

fibrin plug formation

Question 4

bleeding history

Answer 4

has the patient actually got a bleeding disorder
severity
pattern of bleeding
congenital or acquired
mode of inheritance

Question 5

key questions

Answer 5

bruising
epistaxis
post surgical bleeding (important eg teeth out/ tonsillitis)
menorrhagia
post partum haemorrhage
post trauma

Question 6

platelet type bleeding

Answer 6

mucosal
epistacis
purpura
menorrhagia (heavy bleeding)
GI
post surgical
no spontaneuos bleeding to joints

Question 7

Coagulation factor

Answer 7

articular
muscle haematoma
CNS

Question 8

petechia

Answer 8

dont blanch

Question 9

Factor 8 deficiency

Answer 9

haemophilia 8

Question 10

factor 9 deficiency

Answer 10

haemophilia 9 ( 5 times less common)

Question 11

intracranial haemorrhage

Answer 11

rectus sheath haematoma

Question 12

congenital vs acquired

Answer 12

previous episodes
age at first event
associated history

Question 13

haemophilia A &B

Answer 13

X linked
identical phenotypes
severity of bleeding depends on residual coagulation factor activity

Question 14

clinical features

Answer 14

ankle joint
haemarthrosis
muscle haematoma
CNS bleeding
retroperitoneal bleeding
post surgical bleeding

Question 15

haemophilic arthropathy

Answer 15

cartilage degradation

Question 16

clinical complication

Answer 16

synovitis
chronic haemophilic arthropathy
neurovascular compression
stroke

Question 17

diagnosis of haemophilia

Answer 17

clinical
prolonged APTT
normal PT
Reduced FVIII of FIX
genetic analysis

Question 18

treatment

Answer 18

DDA VP
coagulation factor replacement 
recombinant products
transexamic acid
emphasis on prophylaxis

Question 19

complications

Answer 19

viral infection: HIV/ HBV/ HCV
inhibitors Anti FVIII Ab
Rare in FIX
DDAVP contraindicated in heart disease- MI
Hyponatraemia (babies)

Question 20

von Willebrand disease

Answer 20

variable severity
autosomal
platelet type bleeding
quantitative vs qualitative

Question 21

Types

Answer 21

Type 1 quantitative deficiency
type 2 qualitative
type 3 most serious

Question 22

acquired bleeding

Answer 22

thrombocytopenia:
Decreased production: marrow failure, aplasia
Increased production: DIC

Question 23

ITP

Answer 23

Adults vs Children
Associations		Infection esp EBV,HIV
					Collagenosis
					Lymphoma
					Drug induced
Blood isolated thrombocytopenia
Marrow ?
Steroids, IV IgG, Splenectomy, Thrombopoietin analogues (Eltrombopag and romiplostim)

Question 24

Liver disease

Answer 24

Factor I,II,V, VII,VIII,IX,X,XI
prolonged PT, APTT reduced fibrinogen

Cholestasis Vit K dept factor deficiency
Factor II, VII, IX, X.