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LHT2 > Disorders of Thrombosis and Hemostasis CIS > Flashcards

Disorders of Thrombosis and Hemostasis CIS Flashcards

1
Q

primary hemostasis

A
  • platelet adhesion- dep on glycoproteins on platelet surface and mediated by VwF
  • act platelets have storage granules and secrete factors (ADP, serotonin)- which recruit other platelets
  • formation of a platelet plug
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2
Q

secondary hemostasis

A
  • serum coag factors- development of fibrin latticework which braces the platelet plug
  • also recruit platelets
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3
Q

most common cause of bleeding?

A

-thrombocytopenia

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4
Q

lab tests for bleeding disorders

A
  • CBC- for plt count
  • PT- 2, 5, 7, 10, fibrinogen def
  • PTT- 8, 9, 11, 12 def
  • PS examination- morphology of formed elements
  • plt aggregation studies
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5
Q

PT

A
  • extrinsic system
  • 10-13 seconds
  • prolonged- 2, 5, 7, 10, fibrinogen def
  • prolonged in pts taking warfarin or dicoumarol
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6
Q

PTT

A
  • intrinsic system
  • 25-40 seconds
  • prolonged- 8, 9, 11, 12 def
  • prolonged in pts taking heparin
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7
Q

platelet aggregation studies

A
  • determine qualitative platelet defects
  • abnormal in pts taking aspirin or NSAIDS
  • Vonwillebrand disease, storage pool dz, Bernard-soulier syndrome
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8
Q

in pts with petechiae and thrombocytopenia- potential cause?

A

medications!

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9
Q

DIC

A
  • complicaiton of medical, surgical, and obstetrical situations
  • coag systems are act- results in initial thrombosis stage
  • plts and clotting factors depleted- bleeding!
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10
Q

DIC- tx

A

-correction of underlying disorder!!

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11
Q

Thrombotic thrombocytopenic purpura (TTP)

A

-thrombocytopenic purpura, microangiopathic hemolytic anemia, neuro signs, renal dysfxn, and fever
(if add renal failure- HUS!!)

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12
Q

TTP- signs, sx’s

A
  • microangiopathic anemia- schistocytes (RBC fragments)

- hyaline thrombi- occlude capillaries

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13
Q

TTP- 2 forms

A
  • hereditary- mutation of ADAMTS13 gene (von willebrand factor-cleaving protease)
  • acquired- autoab’s to ADAMTS13
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14
Q

TTP- tx

A
  • treat the cause

- plasmapheresis- life saving in 100%

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15
Q

vonWillebrand disease

A
  • dec platelet adhesion to vascular endothelium (usually mediated by vWF)
  • dec or absent production of vWF
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16
Q

vonWillebrand disease- lab, tx

A
  • platelet aggregation tests are normal- esp to ristocetin!!!
  • tx- cryoprecipitate- replaces vWF
  • DDAVP- causes release of vWF from endothelium
17
Q

Factor Deficiencies

A
  • Hemophilia A and B
  • vonwillebrand disease
  • vit K-dep fafctors
18
Q

Hemophilia A

A
  • X-linked recessive
  • def of Factor 8
  • mild (6-25% normal activity); moderate (1-5%); severe (<1%)
19
Q

Hemophilia A- clinical features

A
  • easy bleeding, bruisability
  • hematomas- bleeding into soft tissues and muscles
  • hemarthroses
  • risk for bleeding after surgery
20
Q

Hemophilia B

A
  • def of factor 9

- tx- replacement of factor 9 for hemorrhage or prophylactically for surgery

21
Q

Deficiency of Vit-K dep factors

A
  • bleeding/hemorrhage
  • prolonged PT
  • def of 2, 7, 9, 10, protein C and S
22
Q

Hereditary Hemorrhagic Telangiectasia

A

(Osler-Weber-Rendu syndrome)

  • only endothelial syndrome assoc with hemostatic complications
  • thinning of vessels walls, AV malformations, aneurysmal dilatations thruout body
  • autosomal dominant
  • defect in gene coding for endoglin (CD 105)- a membrane glycoprotein expressed on endo cells
23
Q

Hereditary Hemorrhagic Telangiectasia- clinical features

A
  • telangiectasias- skin, mucous membranes, visceral tissues
  • bleeding- to mild/inapparent trauma; epistaxis most common sx
  • usually benign
24
Q

Hereditary Hemorrhagic Telangiectasia- tx

A

-surgery and laser photoablation of telangiectasias

25
Q

liver dz coagulopathy- treat with?

A

fresh frozen plasma has clotting factors in it)

26
Q

thrombotic disorders

A
  • antithrombin III def
  • protein C and S def
  • factor V leiden syndrome
  • prothrombin 20210
  • antiphospholipid syndrome
  • superficial venous thrombosis
27
Q

AT-III def- clincial

A
  • LE thrombophlebitis and DVT, venous insuff, chronic leg ulcers
  • 50% affected have DVT or PE by age 30
  • inc risk for DVT in pregnant women
  • dx- AT-III levels < 50% of normal activity
28
Q

AT-III Def- tx

A
  • prophylactic tx- anticoagulants
  • pts with DVT- heparin (high doses required)
  • AT-III replacement therapy- pts with DVT who dont respond to heparin
29
Q

Def of protein C and S- clinical, tx

A

(vit K-dep)

  • C- inact factor 5 and 8
  • S- cofactor for protein C
  • clinical- similar to AT-III Def
  • warfarin- dec risk of thromboembolic dz
30
Q

def of protein C and S- most common cause of hypercoagulable state from def of these proteins?

A
  • initiation of warfarin therapy!

- C and S depleted prior to other factors- temporary inc in coagulability

31
Q

Factor V Leiden

A
  • abnormality of factor V at binding site for act protein C (cant be inact)
  • heterozygotes- inc risk for thromboembolic dz
  • homozygotes- high risk for thromboembolism
32
Q

Factor V Leiden- tx

A
  • no prior episodes- monitor, DVT prophylaxis

- prior episodes- lifelong anticoagulation

33
Q

Prothrombin 20210

A
  • inc activity for prothrombin, and inability to de-act prothrombin
  • very high risk of thrombosis
  • tx- same as factor V leiden
34
Q

Antiphospholipid syndrome- terms

A
  • anticardiolipin ab
  • lupus anticoagulant (misnomer)
  • false positive VDRL ab
35
Q

Antiphospholipid syndrome- assoc features

A
  • thromboembolic
  • miscarriage
  • thrombocytopenia
  • cerebral ischemia and recurrent stroke (in young pts_
  • UBO (unidentified bright objects) on MRI scans
  • CT dz- in 50%
  • prolonged PTT- fails to correct with mixing studies
  • valvular HD- in some
  • CAD- in some
36
Q

Antiphospholipid syndrome- dx- 3 tests

A
  • prolonged PTT
  • lack of correction in mixing studies using normal plasma
  • neutralization of inhibitor with excess phospholipid
  • DRVVT (dilute russell viper venom time) may be more specific than PTT test- used for lupus anticoagulant variety!
37
Q

Antiphospholipid syndrome- tx

A
  • no benefit for anticoagulation in those with no hx of thromboembolisms
  • if have hx- lifelong anticoagulation- but most do mult positive tests over a 3-12 month period for dx!
  • anticoagulation during pregnancy- SC heparin
  • hydroxychloroquine- reduce thromboembolism in pts with APS and SLE
38
Q

hypercoagulability panel

A

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

LHT2 (17 decks)

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