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Flashcards in Coag Disorders for Panada's Sheet Deck (79):
1

What will defective platelets increase in terms of the tests?

bleeding time

2

What will defective coagulation affect in terms of the tests?

PT or PTT depending on where the defect is

3

What are two clinical things that are important for thrombophilia?

hypercoagulable state and increased risk for thrombosis

4

What is arterial thrombosis caused by?

a lsion that exposes the subendothelium to platelets in blood

5

What is venous thrombosis caused by?

stasis in blood -- due to immobility and hypercoagulation

6

What does an arterial thrombosis look like?

a white clot b/c high platelet component, associated w/ high blood flow

7

What does a venous thrombosis look like?

a red clot b/c of lower platelets and more fibrin component

8

Is venous thrombosis more fatal then an arterial one?

no

9

What is an arterial thrombosis associated w/?

atrial fibrillation, MI>stroke>PAD

10

How does one treat an arterial thrombosis?

Acute management: Remove clot, stent, aspirin to decrease platelet aggregation, clopidogrel to decrease ADP receptors for aggregation
Chronic -- treat underlying disease

11

What is the Holman Sign?

when one feel pains in the back of the calf during dorsiflexion of the foot b/c DVT clotted veins are painful to stretch

12

What is the common result of DVT?

pulmonary embolism -- SOB is key finding

13

What is the overall goal for DVT?

keep the clot south of the knee

14

What should you think of when there is failure of platelet production?

1. bone marrow failure
2. Megs depression
3. c-MPL receptor mutation

15

What are some causes of increased platelets?

bleeding, splenectomy, inflammatory disease, iron deficiency

16

What is the clinical presentation for thrombocytopenia?

1. spontaneous skin purpura
2. mucosal hemorrhage
3. excess bleeding post-trauma

17

What is ITP?

IgG Abs targeted at platelets causing them to be removed by RES system

18

What can cause ITP?

drug induced ---w/ heparin and PF4 complex formation

19

What is the clinical presentation for ITP?

young women, thrombocytopenia, but no neutropenia or anemia, lots of PETEICHIA!

20

What is the treatment for ITP?

1. common w/ URI in kids -- no treatment
2. adults - give prednisone, IVIg, TPO agonist to make more platelets, and splenectomy

21

What is TTP?

1. congenital -- absence/defective ADAMTS13
2. Acquired - auto-Ab against ADAMTS13

- large vWF-- binds platelets -- occlusion due to aggregation --> microangiopathic anemia

22

What is ADAMTS13?

metalloprotease that cleaves large vWF

23

What is the treatment for TTP?

1. plasma exchange, FFP, steriods, rituximab
2. NEVER GIVE PLATELETS

24

What is the presentation for TTP?

young women, thrombocytopenia, MAHA, neurological symptoms, renal insufficiency, fever

25

What is seen in the PS for TTP?

schistocytes

26

What can a DDx be made w/ for TTP?

1. thromboctyopenia
2. schistocytes/MAHA
3. increased LDH
4. increased indirect bilirubin

27

What causes DIC?

infection, malignancies, OB complications

28

What is the key problem in DIC?

Excess thrombin production --> excess fibrin deposition --> clot formation --> MAHA

29

What is the Rx for DIC?

1. treat underlying cause
2. platelets, FFP, antithrombin 3, activated protein C

30

What is the clinical presentation for DIC?

thromboctopenia, purpura, peripheral gangrene

31

What is the DDx for DIC?

1. low platelets
2. low fibrinogen
3. low clotting factors
4. high D-Dimer ---> KEY TEST FOR DIC

32

What is HHT?

AD, occurs in arterioles/small veins, and platelets are normal

33

What is the clinical presentation for HHT?

easy bruising, spontaneous bleeding from small vessels, mucuosal bleeding

34

What will you commonly see in pts w/ HHT?

bleeding on edge of tongue, inside noses. Look for redspots

35

What is senile purpura?

age-associated atrophy of vasculature so bruising at commonly injured sites

36

What is Ehlers Danlos Syndrome?

defective synthesis of connective tissue

37

What is Henoch Schoeiein Purpura?

IgA mediated vasculitis --> you can feel the purpura!

38

What is bernard soulier?

deficient in GpIb

39

What is Glanzman's Thrombasthenia>

deficient in GpIIb/IIIa

40

What are acquired causes of platelet disease?

1. drugs
2. liver/renal metabolic failure

41

What is the treatment for acquired autoimmune thrombocytopenia due to platelet transfusions?

No treatment option left so be careful

42

What is Hemophilia A?

absence/ lower plasma factor 8, x-linked recessive

43

What is hemophilia B?

absence/ lower plasma factor 9, x-linked recessive

44

What is the clinical presentation for hemophlia A and B?

deep bleeding, bleeding into joints, fusion of joints

45

What is the DDx for hemophilia?

1. long PTTS and normal PT
2. check for inhibitors and then confirm w/ factor assay

46

What is the Rx for hemophilia?

1. EDUCATION
2. local - pressure, topical thrombin
3. system - desmopressin (increase vWF), E-aminocaproic acid (inhibits fibrinolysis)

47

What is vWF Disease?

absent/reduced vWF, AD

48

What are the two functions of vWF?

platelet binding and 8 carrier

49

What is the clinical presentation for vWF disease?

bruising, mucosal bleeding, common when young women begin menstruating

50

What is the DDx for vWF?

1. long PTT, normal PT
2. Measure vWF
3. Longer bleeding time
4. Ristoceitin Test

51

What is the Rx for vWF?

- DDAVP and E-aminocaproic acid

52

What is the DDx for factor 5 mutation?

1. add activated protein C and observe thrombin time. If mutation is present then PT/PTT time will be normal.
2. Screen for mutation

53

What is the Rx for factor 5 mutation?

if no thrombosis, don't start on anticoagulant therapy unless high risk situation (long flights)

54

What is the most common inherited thrombophilia?

factor 5 leiden mutation

55

What presents w/ reurring venous thrombosis starting in early adult life?

inherited antithrombin 3 deficiency

56

What is inherited antithrombin 3 deficiency?

1. AD
2. deficiency in antithrombin 3 so tissue factors aren't activated --> hypercoaguable state

57

What is the test for an antithrombin 3 deficiency?

inject heparin and look at PTT, should be no change in PTT b/c heparin has nothing to bind to

58

What is the Rx for antithrombin 3 deficiency?

antithrombin replacement

59

What is inherited Protein C and S deficiency?

1. AD, hypercoaguable state

60

What is the presenation for protein C and S deficiency?

skin necrosis, thrombosis

61

What is the Rx for protein c/s deficiency?

activated protein C concentrate for replacement

62

What is the inherited prothrombin gene mutation?

G20210A,
1. Sustained generation of thombin
2. increased thrombosis
3. decreased fibrinolysis b/c activated thrombin stimulates secretion of anti-tPA

63

What is the DDx for the prothrombin gene mutation?

1. analyze genetic mutation
2. analyze levels of clotting factors and PT/PTT

64

What is inherited hyperhomocysteinemia?

Bad MTHFR -? no remethylation of THF --> no substrate for the conversion of homocysteine to methionine
- --- bulidup of homocysteine = thrombosis

65

Who is hyperhomocysteinemia common in?

look for unusual thrombosis or CVD in children

66

What are some causes of acquired thrombophilia?

1. ESTROGEN - increased risk for DVT
2. BIRTH CONTROL - increased B-thromboglobulin and decreased antithrombin
3. pregnancy
4. immobilization
5. trauma
6. advanced ago
7. antiphospholip
8. cancer
9.ET
10. nephrotic syndrome

67

What is the anti-phospolipid syndrome?

Abs directed at phospholipid components
1. Anti-cardiolipin
2. antiPS
3. associated proteins: B2GP and thrombin
4. common finiding is lupus anticoagulant

68

What is the clinical presentation for anti-phospholipid syndrome?

1. thrombosis
2. fetal loss in 2nd semester
3. dermatological issues
4. blurred vision
5. stroke
6. Thrombosis w/ anticoagulant lupus but no SLE diagnosis

69

What is the DDx for anti-phospholipid syndrome?

1. high PTT is not corrected, shows inhibitor of coagulation, no deficiency in clotting factor
2. corrected in hexagonal phase b/c it competes for binding of lupus anticoagulant
3. Russell Viper Venom Test

70

What does the russell viper venom test test for?

factors 10 and 5

71

What is the HIT syndrome?

1. autoAb develops for heparin-PF4 complex
2. Ab and complex binds to platletes via platelets Fc receptor
3. causes platelet aggregation and activation

72

What is the clinical presentation of HIT?

- thrombosis but not bleeding

73

What will labs show for HIT?

1. thrombosis -- aggregation of platelets
2. thrombocytopenia -- increased use of platelets

74

What is the DDx for HIT?

look for anti-Heparin/PF4 Ab via ELISA

75

What is the Rx for HIT?

take pt off heparine, give DTI
- don't give DTI via IV if kidney disease

76

What is coumarin/coumadin induced necrosis?

1. Warfarin therapy has immediate effects in liver but delayed systemic effects b/c circulating clotting factors must be cleared
2. systemic effect take 1 wek
3. anti-coagulation factors, PC and PS, have shorter half lives. Therefore, inhibition of PC/PS formation generates clots
4. Results in tissue necrosis

77

What are effects of coumadin?

tissue necrosis of skin/fatty areas

78

What is the Rx for warfarin induced necrosis?

1. remove warfarin
2. administer Vit K
3. administer prothrombin complex (replace all Vit K factors including PC/PS
4. FFP - replace all clotting factors
5. recombinant 7a

79

How do you prevent wafarin induced necrosis?

give heparin and warfarin at same time and wean off heparin