Coag Disorders for Panada's Sheet Flashcards

1
Q

What will defective platelets increase in terms of the tests?

A

bleeding time

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2
Q

What will defective coagulation affect in terms of the tests?

A

PT or PTT depending on where the defect is

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3
Q

What are two clinical things that are important for thrombophilia?

A

hypercoagulable state and increased risk for thrombosis

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4
Q

What is arterial thrombosis caused by?

A

a lsion that exposes the subendothelium to platelets in blood

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5
Q

What is venous thrombosis caused by?

A

stasis in blood – due to immobility and hypercoagulation

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6
Q

What does an arterial thrombosis look like?

A

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

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7
Q

What does a venous thrombosis look like?

A

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

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8
Q

Is venous thrombosis more fatal then an arterial one?

A

no

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9
Q

What is an arterial thrombosis associated w/?

A

atrial fibrillation, MI>stroke>PAD

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10
Q

How does one treat an arterial thrombosis?

A

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

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11
Q

What is the Holman Sign?

A

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

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12
Q

What is the common result of DVT?

A

pulmonary embolism – SOB is key finding

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13
Q

What is the overall goal for DVT?

A

keep the clot south of the knee

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14
Q

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

A
  1. bone marrow failure
  2. Megs depression
  3. c-MPL receptor mutation
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15
Q

What are some causes of increased platelets?

A

bleeding, splenectomy, inflammatory disease, iron deficiency

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16
Q

What is the clinical presentation for thrombocytopenia?

A
  1. spontaneous skin purpura
  2. mucosal hemorrhage
  3. excess bleeding post-trauma
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17
Q

What is ITP?

A

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

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18
Q

What can cause ITP?

A

drug induced —w/ heparin and PF4 complex formation

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19
Q

What is the clinical presentation for ITP?

A

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

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20
Q

What is the treatment for ITP?

A
  1. common w/ URI in kids – no treatment

2. adults - give prednisone, IVIg, TPO agonist to make more platelets, and splenectomy

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21
Q

What is TTP?

A
  1. congenital – absence/defective ADAMTS13
  2. Acquired - auto-Ab against ADAMTS13
  • large vWF– binds platelets – occlusion due to aggregation –> microangiopathic anemia
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22
Q

What is ADAMTS13?

A

metalloprotease that cleaves large vWF

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23
Q

What is the treatment for TTP?

A
  1. plasma exchange, FFP, steriods, rituximab

2. NEVER GIVE PLATELETS

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24
Q

What is the presentation for TTP?

A

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

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25
Q

What is seen in the PS for TTP?

A

schistocytes

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26
Q

What can a DDx be made w/ for TTP?

A
  1. thromboctyopenia
  2. schistocytes/MAHA
  3. increased LDH
  4. increased indirect bilirubin
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27
Q

What causes DIC?

A

infection, malignancies, OB complications

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28
Q

What is the key problem in DIC?

A

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

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29
Q

What is the Rx for DIC?

A
  1. treat underlying cause

2. platelets, FFP, antithrombin 3, activated protein C

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30
Q

What is the clinical presentation for DIC?

A

thromboctopenia, purpura, peripheral gangrene

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31
Q

What is the DDx for DIC?

A
  1. low platelets
  2. low fibrinogen
  3. low clotting factors
  4. high D-Dimer —> KEY TEST FOR DIC
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32
Q

What is HHT?

A

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

33
Q

What is the clinical presentation for HHT?

A

easy bruising, spontaneous bleeding from small vessels, mucuosal bleeding

34
Q

What will you commonly see in pts w/ HHT?

A

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

35
Q

What is senile purpura?

A

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

36
Q

What is Ehlers Danlos Syndrome?

A

defective synthesis of connective tissue

37
Q

What is Henoch Schoeiein Purpura?

A

IgA mediated vasculitis –> you can feel the purpura!

38
Q

What is bernard soulier?

A

deficient in GpIb

39
Q

What is Glanzman’s Thrombasthenia>

A

deficient in GpIIb/IIIa

40
Q

What are acquired causes of platelet disease?

A
  1. drugs

2. liver/renal metabolic failure

41
Q

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

A

No treatment option left so be careful

42
Q

What is Hemophilia A?

A

absence/ lower plasma factor 8, x-linked recessive

43
Q

What is hemophilia B?

A

absence/ lower plasma factor 9, x-linked recessive

44
Q

What is the clinical presentation for hemophlia A and B?

A

deep bleeding, bleeding into joints, fusion of joints

45
Q

What is the DDx for hemophilia?

A
  1. long PTTS and normal PT

2. check for inhibitors and then confirm w/ factor assay

46
Q

What is the Rx for hemophilia?

A
  1. EDUCATION
  2. local - pressure, topical thrombin
  3. system - desmopressin (increase vWF), E-aminocaproic acid (inhibits fibrinolysis)
47
Q

What is vWF Disease?

A

absent/reduced vWF, AD

48
Q

What are the two functions of vWF?

A

platelet binding and 8 carrier

49
Q

What is the clinical presentation for vWF disease?

A

bruising, mucosal bleeding, common when young women begin menstruating

50
Q

What is the DDx for vWF?

A
  1. long PTT, normal PT
  2. Measure vWF
  3. Longer bleeding time
  4. Ristoceitin Test
51
Q

What is the Rx for vWF?

A
  • DDAVP and E-aminocaproic acid
52
Q

What is the DDx for factor 5 mutation?

A
  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
Q

What is the Rx for factor 5 mutation?

A

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

54
Q

What is the most common inherited thrombophilia?

A

factor 5 leiden mutation

55
Q

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

A

inherited antithrombin 3 deficiency

56
Q

What is inherited antithrombin 3 deficiency?

A
  1. AD

2. deficiency in antithrombin 3 so tissue factors aren’t activated –> hypercoaguable state

57
Q

What is the test for an antithrombin 3 deficiency?

A

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

58
Q

What is the Rx for antithrombin 3 deficiency?

A

antithrombin replacement

59
Q

What is inherited Protein C and S deficiency?

A
  1. AD, hypercoaguable state
60
Q

What is the presenation for protein C and S deficiency?

A

skin necrosis, thrombosis

61
Q

What is the Rx for protein c/s deficiency?

A

activated protein C concentrate for replacement

62
Q

What is the inherited prothrombin gene mutation?

A

G20210A,

  1. Sustained generation of thombin
  2. increased thrombosis
  3. decreased fibrinolysis b/c activated thrombin stimulates secretion of anti-tPA
63
Q

What is the DDx for the prothrombin gene mutation?

A
  1. analyze genetic mutation

2. analyze levels of clotting factors and PT/PTT

64
Q

What is inherited hyperhomocysteinemia?

A

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

65
Q

Who is hyperhomocysteinemia common in?

A

look for unusual thrombosis or CVD in children

66
Q

What are some causes of acquired thrombophilia?

A
  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
Q

What is the anti-phospolipid syndrome?

A

Abs directed at phospholipid components

  1. Anti-cardiolipin
  2. antiPS
  3. associated proteins: B2GP and thrombin
  4. common finiding is lupus anticoagulant
68
Q

What is the clinical presentation for anti-phospholipid syndrome?

A
  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
Q

What is the DDx for anti-phospholipid syndrome?

A
  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
Q

What does the russell viper venom test test for?

A

factors 10 and 5

71
Q

What is the HIT syndrome?

A
  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
Q

What is the clinical presentation of HIT?

A
  • thrombosis but not bleeding
73
Q

What will labs show for HIT?

A
  1. thrombosis – aggregation of platelets

2. thrombocytopenia – increased use of platelets

74
Q

What is the DDx for HIT?

A

look for anti-Heparin/PF4 Ab via ELISA

75
Q

What is the Rx for HIT?

A

take pt off heparine, give DTI

- don’t give DTI via IV if kidney disease

76
Q

What is coumarin/coumadin induced necrosis?

A
  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
Q

What are effects of coumadin?

A

tissue necrosis of skin/fatty areas

78
Q

What is the Rx for warfarin induced necrosis?

A
  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
Q

How do you prevent wafarin induced necrosis?

A

give heparin and warfarin at same time and wean off heparin