Haemostasis and thrombosis 2

Question 1

which is the most common haemophilia

Answer 1

haemophilia A

Question 2

what is the deficiency in haemophilia A

Answer 2

factor 8

Question 3

features haemophilia A

Answer 3

severe spontaneous bleeding especially into joints- haemarthroses and muscles; onset in early childhood; chronic debilitating joint disease; pseudotumours

Question 4

lab features haem A

Answer 4

PT normal, APTT incr, factor 8 decr, vwf normal

Question 5

treatment haemophilia A

Answer 5

infusions factor 8- elevate platelets level to 20-50% normal for severe bleeding. desmopressin

Question 6

inheritance haemophilia

Answer 6

sex linked

Question 7

which factor is deficient in haemophilia B

Answer 7

9

Question 8

inheritance haem B

Answer 8

sex linked. 4x less common than A, milder than A

Question 9

treatment haemo B

Answer 9

factor 9 concentrate. desmopressin not effective

Question 10

how is VWF disease inherited

Answer 10

autosomal dominant

Question 11

role of VWF

Answer 11

carries factor 8in plasma and mediates platelet adhesion to endothelium.

Question 12

if VWF or haemophilia A more common

Answer 12

VWF disease

Question 13

features VWF disease

Answer 13

bleeding- from mucous membranes- mouth, epistaxis, menorrhagia. haemarthroses rare

Question 14

lab findings in VWF

Answer 14

incr APTT, normal PT, factor 8 and VWF decr

Question 15

treatment vwf

Answer 15

intermediate purity factor 8 concentrate- contains VWF and factor 8. if severe bleeding- high purity VWF concentrates. desmopressin, tranexamic acid

Question 16

what can cause acquired disorders of coagulation

Answer 16

liver disease- defects in coagulation, platelets and fibrinolysis

Question 17

what may reduced levels proteins C and S, anti thrombin and alpha2- antiplasmin lead to

Answer 17

DIC

Question 18

what may dysfibrinogenaemia lead to

Answer 18

haemorrhage or thrombosis

Question 19

what happens in DIC

Answer 19

release procoagulant material into circulation or endothelial damage- generalised activation coag and fibrinolytic pathways- widespread fibrin deposition

Question 20

causes DIC

Answer 20

infection- septicaemia, meningitis, malaria; malignancy- promyelocytic leukaemia; obs- septic abortion, pre eclampsia, retained products of conception, amniotic fluid embolism; shock; hypersensitivity; burns; trauma

Question 21

why do you get thrombosis and bleeding in DIC

Answer 21

as the coagulation process is consuming clotting factors and platelets normal clotting is disrupted so get severe bleeding

Question 22

lab results DIC

Answer 22

platelets decr, PT incr, APTT incr, fibrinogen decr

Question 23

treatment DIC

Answer 23

platelets if low platelets, cryoprecipitate if low fibrinogen, FFP to replace coag factors

Question 24

if thrombosis predominates- DIC

Answer 24

anticoag?

Question 25

effects DIC

Answer 25

widespread bleeding and thrombosis, acute resp distress syndrome, renal failure, liver dysfunction, GI bleeding, vaginal bleeding, skin ecchymoses and purpura, TE, gangrene

Question 26

role of vitamin K

Answer 26

activate factors 2,7,9,10, and proteins C and S

Question 27

what could lead to vitamin K deficiency

Answer 27

diet, broad spec antibios, biliary tract disease, intestinal malabsorption

Question 28

why do newborns have haemorrhagic disease

Answer 28

immaturity of liver and low levels vit K. give IM vit K

Question 29

what happens if patient has severe epistaxis but you check platelets and theyre normal

Answer 29

platelet function defect. treat with platelets

Question 30

cause platelet function defect- inherited

Answer 30

inherited- Bernard-Soulier, Glanzmann, storage pool disease, VWF.

Question 31

causes platelet function defect- acquired

Answer 31

drugs- aspirin, NSAIDs; myeloproliferative; uraemia; paraproteinaemia

Question 32

what is dipyridamole

Answer 32

phosphodiesterase inhibitor- raises platelet AMP reducing their sensitivity to activating stimuli

Question 33

what does fibrinolytic therapy do

Answer 33

enhance conversion plasminogen to plasmin which degrades fibrin

Question 34

what does heparin do

Answer 34

activate antithrombin, impairs platelet function

Question 35

why does heparin lead less to bleeding

Answer 35

greater ability to inactivate factor Xa less of an effect on thrombin and platelets

Question 36

if LMWH needs monitoring (not usually)

Answer 36

factor Xa assay

Question 37

how long does it take to fully coagulate starting warfarin

Answer 37

48-72 hours

Question 38

what do you need to maintain INR at

Answer 38

2-3 but if high risk eg mechanical heart valves maintain at 2.5-3.5

Question 39

how much vit K to give if haemorrhage

Answer 39

10mg IV