Bleeding Disorders

Question 1

What are hte causes of hereditary vascualr abnormalities assoc. with priamry haemostasis issues?

Answer 1

Marfans; Ehler Danlos

Question 2

What are the acquired causes of vascular abnormalities associated with priamry haemostasis problems?

Answer 2

vasculitis; old age; scurvy; steroids

Question 3

Where is bleeding primary haemostasis seen?

Answer 3

mucous membranes andl ower limb

Question 4

What is often the trigger for autoimmune thrombocytopenic purpura?

Answer 4

often viral: HIV; EBV

Question 5

What are the causes of thrombocytopenia?

Answer 5

reduced production: marrow issue; increased destruction

Question 6

what are the causes of increased destruction in thrombocyopenia?

Answer 6

IPD; DIC; hypersplenism

Question 7

What are the causes of platelet functional defects?

Answer 7

drugs: aspiring; NSAIDs; renal failure

Question 8

Why would von Willebrand factor deficiency lead to increase in APTT?

Answer 8

carries around factor VIII, so will have less VIII for the intrinsic pathway

Question 9

What is the commonest cause of primary haemostatic failure?

Answer 9

thrombocytopenia

Question 10

What are the causes of failure of fibrin clot formation?

Answer 10

multiple clotting factor deficiencies- acquried; single factor deficiency: haemophilia

Question 11

What are the causes of multiple factor deficiencies?

Answer 11

liver failure; vit K deficiency/warfarin; DIC

Question 12

What are the abnoramlities on blood tests with multiple factor deficiens?

Answer 12

prolonged PT and APTT

Question 13

Where are coag factors synthesised?

Answer 13

in hepatocytes

Question 14

What coag factors are carboxylated by Vit K?

Answer 14

II; VII; IX and X

Question 15

What are the sources of vitamin K?

Answer 15

diet (green leafy veg); intestinal bacterial synthesis

Question 16

What type of vitamin is Vit K?

Answer 16

fat-soluble

Question 17

Where is vit K absorbed?

Answer 17

upper intestine

Question 18

What are the causes of vit K deficiency?

Answer 18

poor dietary intake; malabsorption; obstructive jaundice; warfarin; haemorrhagic disease of the newborn

Question 19

What is DIC?

Answer 19

excessive and inappropriate activation of the haemostatic system

Question 20

What happens in DIC?

Answer 20

microvascular thrombus formation; clotting factor consumption

Question 21

why are newborns vit k deficient?

Answer 21

no dietary vit K (very little in breast milk); no intestinal flora to synthesise; imature liver

Question 22

What are the causes of DIC?

Answer 22

sepsis; obstetric emergencies; malignancy; hypovolaemic shock; multiple traum

Question 23

What is the treatment for DIC?

Answer 23

treat underlying cause; platelets; plasma; fibrinogen

Question 24

What is the inheritance of haemophilias?

Answer 24

x-linked

Question 25

What is haemophilia A?

Answer 25

factor VIII deficiency

Question 26

What is haemophilia B?

Answer 26

factor IX deficinecy

Question 27

Which type of blood vessels is there typically bleeding from in haemophilia?

Answer 27

medium-large blood vessels

Question 28

Which coag factor has the shortest half0life?

Answer 28

VII

Question 29

Which test of fibrin clot formation is suually abnormal first in DIC?

Answer 29

PT then APTT

Question 30

Which blood test is abnormal in haemophilia?

Answer 30

APTT

Question 31

What are the clinical features of haemophilia?

Answer 31

recurrent haemarthroses; recurrent soft tissue bleeds; proonged bleeding after procedures