bleeding disorders

Question 1

disorders causing high platelet count

Answer 1

essential thrombocythaemia

other MDPs

Question 2

disorders causing low platelet count

Answer 2

bone marrow failure (aplastic anaemia, malignancy, chemotherapy drugs)
platelets are used up (DIC, HUS, TTP)
ITP (autoantibody against platelets)
hypersplenism (liver cirrhosis)

Question 3

microvascular bleeding but normal platelets

Answer 3

collagen abnormalities (marfans, ehlers danlos, elderly, Vit C deficiency - scurvy)
VWF deficiency
henoch schonlein
renal disease (increased uric acid interferes with platelet function)
anti-platelet drugs (aspirin)

Question 4

causes of increased PTT

Answer 4

multi clotting factor deficiency:
vit K deficiency
warfarin 
DIC
liver failure

Question 5

causes of increased APTT

Answer 5

single clotting factor deficiency:
haemophilia A (factor 8)
haemophilia B (factor 9)VWF deficiency (VWF carries factor 8)
liver failure
Vit K deficiency/warfarin
DIC

Question 6

how do disorders of platelets/vasculature present

Answer 6

microvascular bleeds - bruising, purpura, petechiae

mucosal bleeds - gum bleeding, nose bleeds

Question 7

how do diseases of clotting factors present

Answer 7

bleeding into large joints - swollen

Question 8

what is disseminated intravascular coagulation

Answer 8

excessive activation of primary, secondary and fibrinolytic haemostasis

Question 9

pathophysiology of DIC

Answer 9

microvascular thrombus formation causing end organ failure

uses up resources - less platelets and clotting factors (risk of bleeding)

Question 10

risk factors for DIC

Answer 10

blood transfusion reaction
malignancy (especially leukaemia)
pancreatitis
sepsis
liver disease 
pregnancy
surgery 
burns 
haemangioma

Question 11

presentation of DIC

Answer 11

microvascular bleeds
bleeding that starts spontaneously - from wounds/surgery scars/drainage sites etc
spontaneous bruising 
hypotension
renal failure

Question 12

bloods in DIC

Answer 12

abnormal everything: 
low platelets 
high PTT and APTT
low fibrinogen 
high FDPs (Ddimers)

Question 13

what is seen on a blood film in DIC

Answer 13

schistocytes

Question 14

what investigation can rule out DIC

Answer 14

Ddimers - if negative, its not DIC

Question 15

management of DIC

Answer 15

platelet replacement
fresh frozen plasma (cryoprecipitate) to replace clotting factors
fibrinogen (increases fibrinolysis)

Question 16

inheritance if haemophilia A and B

Answer 16

X linked recessive

Question 17

what is more common, haemophilia A or B

Answer 17

A

Question 18

presentation of haemophilia

Answer 18

recurrent haemarthroses
recurrent soft tissue bleeds
prolonged bleeding after dental extractions or surgery

Question 19

blood results in haemophilia

Answer 19

increase in APTT only

Question 20

management of haemophilia

Answer 20

avoid NSAIDs

if severe, specific clotting factor infusion

Question 21

management for ITP

Answer 21

immunosuppression