Hubbard thrombosis and hemostasis Flashcards

1
Q

what is the most common cause of bleeding

A

thrombocytopenia

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2
Q

Normal PT?
tests what?
prolonged in what deficiencies?
prolonged with what drugs

A
  • 10-13 seconds
  • extrinsice system
  • factors II, V, VII, X, and fibrinogen
  • Warfarin or dicoumarol
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3
Q

Normal PTT?
test what?
Prolonged in what deficiencies?
prolonged with what drug?

A
  • 25-40 seconds
  • extrinsic
  • factors VIII, IX, XI, XII
  • Heparin
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4
Q

What is used to determine platelet abnormalities when platelets are normal in numbers (qualitative platelet defects)?
used for what diseases?

A

platelet aggregation studies

  • vWB disease
  • Bernard-Soulier syndrome
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5
Q

In patients with petechiae and thrombocytopenia, if you offer up the possibility of what causing it, you will look like a rockstar

A

medications

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6
Q

DIC is a complication of what situations?

A
  • medical
  • surgical
  • obstetrical
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7
Q
  • intrinsice and extrinsic coagulation systems are activation
  • thrombin escapes (initial thrombosis stage may not be seen)
  • platelets and clotting factors are depleted, causing bleeding
A

DIC

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8
Q

Treatment of DIC

A
  • correction of underlying disorder–sepsis, bowel obstruction, etc
  • Heparin .. not usually used unless overt thrombosis occurs
  • supportive care–platelet and factor replacement
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9
Q

Microangiopathic hemolytic anemia, thrombocytopenia (<50,000), fever, neurological symptoms?
add renal failure to this?

A

TTP

HUS

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10
Q
  • Schistocytes
  • helmet cells
  • “Waring blender” effect
A

TTP and HUS

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11
Q

pathologic lesion are hyaline thrombi which occlude the capillaries of virtually every organ in the body

A

TTP and HUS

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12
Q

2 forms of TTP

A
  • hereditary mutation of ADAMTS13 gene

- acquired- autoantibodies directed at ADAMTS13

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13
Q

Treatment ofTTP

A

Plasmaphoresis–life saving in virtually 100% of cases

-Mortality virtually 100% if not treated

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14
Q

platelet aggregation tests abnormal, especially to ristocetin

A

vonWillebrand Disease

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15
Q

Treatment of vonWillebrand Disease

A
  • Cryoprecipitate replaces vWF

- DDAVP causes release of vWF from endothelium

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16
Q

inheritance of Hemophilia A and B

A

X linked recessive

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17
Q

classifications of severity in hemophilia A

A
  • mild (6-25% normal activity)
  • moderate (1-5% normal activity)
  • Severe (<1%)
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18
Q

Clinical features of hemophilia

A
  • easy bleeding and bruisability
  • hematomas from bleeding into soft tissue and muscles
  • Hemarthroses
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19
Q

factors for Hemophilia A and B

A

VIII and IX respectively

20
Q

characteristics of deficiency of vitamin K and dependent factors

A
  • bleeding/hemorrhage
  • Prolonged PT
  • deficiency of factors II, VII, IX, X, protein C and S
21
Q

What is the only endolthelial syndrome associated with hemostatic complications

A

-Hereditary hemorrhagic telangiectasia aka Osler-Weber-Rendu syndrome

22
Q
  • inheritance of hereditary hemorrhagic telangiectasia?
  • defect in what gene?
  • what chromosome
A
  • autosomal dominant
  • endoglin (CD 105) . a membrane glycoprotein expressed on endolthelial cells
  • chromosome 9
23
Q

symptoms of hereditary hemorrhagic telangiectasia

A
  • epistaxis (most frequent)
  • telangiectasias gradually apprering throughout life located in skin, mucous membranes, and visceral tissues
  • bleeding to mild or inapparent trauma
24
Q

course and treatment of hereditary hemorrhagic telangiectasia

A
  • usually benign clinical course
  • recurrent bleeds frequent but death from exsanguination is rare
  • surgery and laser photoablation of telangiectasias of some value but care must be used in selecting site
25
Q

normal bilirubin

A

.4 IU/dL

26
Q

what organ can cause coagulopathy if severely damaged

A

liver . . think alcoholics or hepatitis or cirrhosis

27
Q

what can be given to replace clotting factors?

A

fresh frozen plasma

28
Q

50% of these patients have DVT and/or PE by age 30

A

AT-III deficiency

29
Q

what is the normal job of anti-thrombin III

A

to slow progression from prothombin to thrombin

30
Q

AT-III deficiency and pregnancy

A

significantly increased risk for DVT in pregnant women due to pregnancy induced hypercoagulability

31
Q

Diagnosis of AT-III deficiency

A

diminshed levels of AT-III in serum (<50% normal activity)

32
Q

Treatment of AT-III deficiency

A
  • prophylactic treatement with anticoagulants
  • patients with DVT should recieve heparin but much higher doses
  • AT-III replacement is available for known AT-III deficient patients with DVT who do not respond initially to heparin
33
Q

explain what protein C and S do

A
  • both vitamin K dependent
  • Protein C inactivated factors V and VIII
  • protein S is a cofactor for protein C
34
Q

What is used in patients with a deficiency of protein C and S to decrease the risk of thromboembolic disease

A

Warfarin

35
Q

What is the most common cause of hypercoagulable state from deficiency of protein C and S

A
  • initiation of warfarin therapy

- Proteins C and S are depleted prior to other factors, resulting in a temporary increase in coagulability

36
Q

Abnormality of factor V at binding site for activated protein C

A

Factor V leiden

37
Q

treatment of Factor V leiden

A
  • no prior episodes: monitor; DVT prophylaxis and risk reduction
  • prior episodes: consider lifelone anticoagulation
38
Q

G-A mutation resulting in increased activity for prothrombin and inability to de-activate prothrombin

A

prothrombin 20210 . . . treatment same as factor V leiden

39
Q

associated features with antiphospholipid syndrome

A
  • Thromboembolic phenomena
  • MISCARRIAGE
  • thrombocytopenia
  • cerebral ischemia and recurrent stroke (especially in young patients)
  • UBOs: unidentified bright objects on MRIs
40
Q

other associated features with antiphospholipid syndrome

A
  • Connect tissue disease in >50%
  • Prolonged PTT that fails to correct with mixing studies
  • Valvular heart disease in some
  • CAD in some
41
Q

Diagnosis of Antiphospholipid syndrome

A
  • Prolonged PTT
  • Lack of correction in mixing studies
  • Neutralization of inhibitor with excess phospholipid
42
Q

What test can be used that is more specific in diagnosing antiphospholipid syndrome than PTT

A

DRVVT- Dilute Russell Viper Venom Time

43
Q

General Treatment of antiphospholipid syndrome

A
  • no benefit for anticoagulation in those WITHOUT a history of thromboembolic disease
  • WITH history: lifelong anticoagulant (warfarin)–don’t base on single test. need multiple positive tests over a 3-12 month period to make diagnosis
44
Q

specific treatment for antiphospholipid syndrome

A
  • pregnancy: SC heparin

- patients with SLE: hydroxychloroquine

45
Q

What is included in a hypercoagulability panel?

A
  • factor V leiden
  • prothrombin gene mutation
  • protein C and S