Haemostasis and thrombosis 2 Flashcards Preview

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Flashcards in Haemostasis and thrombosis 2 Deck (39):
1

which is the most common haemophilia

haemophilia A

2

what is the deficiency in haemophilia A

factor 8

3

features haemophilia A

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

4

lab features haem A

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

5

treatment haemophilia A

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

6

inheritance haemophilia

sex linked

7

which factor is deficient in haemophilia B

9

8

inheritance haem B

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

9

treatment haemo B

factor 9 concentrate. desmopressin not effective

10

how is VWF disease inherited

autosomal dominant

11

role of VWF

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

12

if VWF or haemophilia A more common

VWF disease

13

features VWF disease

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

14

lab findings in VWF

incr APTT, normal PT, factor 8 and VWF decr

15

treatment vwf

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

16

what can cause acquired disorders of coagulation

liver disease- defects in coagulation, platelets and fibrinolysis

17

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

DIC

18

what may dysfibrinogenaemia lead to

haemorrhage or thrombosis

19

what happens in DIC

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

20

causes DIC

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

21

why do you get thrombosis and bleeding in DIC

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

22

lab results DIC

platelets decr, PT incr, APTT incr, fibrinogen decr

23

treatment DIC

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

24

if thrombosis predominates- DIC

anticoag?

25

effects DIC

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

26

role of vitamin K

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

27

what could lead to vitamin K deficiency

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

28

why do newborns have haemorrhagic disease

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

29

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

platelet function defect. treat with platelets

30

cause platelet function defect- inherited

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

31

causes platelet function defect- acquired

drugs- aspirin, NSAIDs; myeloproliferative; uraemia; paraproteinaemia

32

what is dipyridamole

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

33

what does fibrinolytic therapy do

enhance conversion plasminogen to plasmin which degrades fibrin

34

what does heparin do

activate antithrombin, impairs platelet function

35

why does heparin lead less to bleeding

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

36

if LMWH needs monitoring (not usually)

factor Xa assay

37

how long does it take to fully coagulate starting warfarin

48-72 hours

38

what do you need to maintain INR at

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

39

how much vit K to give if haemorrhage

10mg IV