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

- hereditary mutation of ADAMTS13 gene - acquired- autoantibodies directed at ADAMTS13

Hubbard thrombosis and hemostasis Flashcards by Garrett Gilbert

what is the most common cause of bleeding

thrombocytopenia

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

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

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

25-40 seconds
extrinsic
factors VIII, IX, XI, XII
Heparin

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

platelet aggregation studies

vWB disease
Bernard-Soulier syndrome

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

medications

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DIC is a complication of what situations?

medical
surgical
obstetrical

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intrinsice and extrinsic coagulation systems are activation
thrombin escapes (initial thrombosis stage may not be seen)
platelets and clotting factors are depleted, causing bleeding

DIC

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Treatment of DIC

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

TTP

HUS

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Schistocytes
helmet cells
“Waring blender” effect

TTP and HUS

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

TTP and HUS

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2 forms of TTP

hereditary mutation of ADAMTS13 gene

- acquired- autoantibodies directed at ADAMTS13

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Treatment ofTTP

Plasmaphoresis–life saving in virtually 100% of cases

-Mortality virtually 100% if not treated

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platelet aggregation tests abnormal, especially to ristocetin

vonWillebrand Disease

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Treatment of vonWillebrand Disease

Cryoprecipitate replaces vWF

- DDAVP causes release of vWF from endothelium

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inheritance of Hemophilia A and B

X linked recessive

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classifications of severity in hemophilia A

mild (6-25% normal activity)
moderate (1-5% normal activity)
Severe (<1%)

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Clinical features of hemophilia

easy bleeding and bruisability
hematomas from bleeding into soft tissue and muscles
Hemarthroses

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factors for Hemophilia A and B

VIII and IX respectively

characteristics of deficiency of vitamin K and dependent factors

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

What is the only endolthelial syndrome associated with hemostatic complications

-Hereditary hemorrhagic telangiectasia aka Osler-Weber-Rendu syndrome

inheritance of hereditary hemorrhagic telangiectasia?
defect in what gene?
what chromosome

autosomal dominant
endoglin (CD 105) . a membrane glycoprotein expressed on endolthelial cells
chromosome 9

symptoms of hereditary hemorrhagic telangiectasia

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

course and treatment of hereditary hemorrhagic telangiectasia

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

normal bilirubin

.4 IU/dL

what organ can cause coagulopathy if severely damaged

liver . . think alcoholics or hepatitis or cirrhosis

what can be given to replace clotting factors?

fresh frozen plasma

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

AT-III deficiency

what is the normal job of anti-thrombin III

to slow progression from prothombin to thrombin

AT-III deficiency and pregnancy

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

Diagnosis of AT-III deficiency

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

Treatment of AT-III deficiency

- 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

explain what protein C and S do

- both vitamin K dependent - Protein C inactivated factors V and VIII - protein S is a cofactor for protein C

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

Warfarin

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

- initiation of warfarin therapy | - Proteins C and S are depleted prior to other factors, resulting in a temporary increase in coagulability

Abnormality of factor V at binding site for activated protein C

Factor V leiden

treatment of Factor V leiden

- no prior episodes: monitor; DVT prophylaxis and risk reduction - prior episodes: consider lifelone anticoagulation

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

prothrombin 20210 . . . treatment same as factor V leiden

associated features with antiphospholipid syndrome

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

other associated features with antiphospholipid syndrome

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

Diagnosis of Antiphospholipid syndrome

- Prolonged PTT - Lack of correction in mixing studies - Neutralization of inhibitor with excess phospholipid

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

DRVVT- Dilute Russell Viper Venom Time

General Treatment of antiphospholipid syndrome

- 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

specific treatment for antiphospholipid syndrome

- pregnancy: SC heparin | - patients with SLE: hydroxychloroquine

What is included in a hypercoagulability panel?

- factor V leiden - prothrombin gene mutation - protein C and S