CIS: thrombosis and Hemostasis Flashcards

1
Q

what is cocktail purpura

A

when ppl drink gin and tonics and then have petechiae under their eyes with a low platelete count
-the quinine in the tonic causes the platelets to clump and be removed from circulation

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2
Q

What is the most common cause of bleeding

A

thrombocytopenia

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3
Q

what does the PTT do

A

asses the intrinsic system

  • norm is 25-40 sec
  • prolonged in deficiencies of 8,9,11,12
  • prolonged in pts on heparin
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4
Q

What is the peripheral smear examination?

A

evaluates morphology of the formed elements of the blood as well as the numbers of elements present

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5
Q

What could be a cause of thrombocytopenia in a patient?

A

the medications they’re using

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6
Q

DIC

A

complication of medical, surgical, and obstetrical situations

  • coag systems are actived (ext and intrinsic)
  • initial thrombosis, short lived, we don’t see it clinically
  • as platelets and clotting factors are depleted, bleeding ensues, which is the major feature of the disease
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7
Q

How to tx DIC

A
  • correction of underlying disorder
  • Heparin, not usually used unless over thrombosis occurs
  • supportive care, platelet and factor replacement
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8
Q

Thrombotic Throbocytopenic purpura

A
  • thrombocytopenic purpura
  • microangiopathic anemia
  • fluctuating neurological signs
  • renal dysfunction
  • febrility
  • just add renal failure and it’s HUS
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9
Q

What does the Thrombin time (TT) do?

A

assesses for abnormalities or deficiency of fibrinogen

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10
Q

ADAMTS13

A

it cleaves vW factor

  • mutation in this gives us the hereditary form of TTP
  • Acquired forms come from autoantibodies to ADAMTS13
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11
Q

Tx of TTP

A

-plasmapheresis: life saving in virtually 100% of cases

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12
Q

What will TTP look like?

A
  • Microangiopathic anemia
  • schistocytes, helmets… that is the Waring blender effect
  • Pathologic lesion: hyaline thrombi which occlude the capillaries of virtually every organ in the body
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13
Q

vonWillebrand disease

A
  • very large group of related diseases that have their hallmark feature decreased platelet adhesion to the vascular endothelium as mediated by vW factor
  • in most cases, the pathologic lesion is decreased or absent production of vWF
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14
Q

tx of vonWillebrand disease

A
  • cryoprecipitate: replaces vWF

- DDAVP- causes release of vWF from endothelium

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15
Q

In what diseases will we see defects in platelet function?

A
  • Uremia: impaired platelet adhesion
  • Dysproteinemias: interference with platelet membrane function
  • Autoimmune disorders: multiple abnormalities
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16
Q

What diseases will give us hemorrhage related to factor deficiency?

A
  • Hemophila A and B
  • vW disease
  • Vit K dependent factors
  • usually a prolonged coagulation times (PT,PTT)
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17
Q

Hemophilia A

A
  • X linked recessive trait characterized by a deficiency of factor 8
  • risk of bleeding corresponds to degree of deficiency
  • mild (6-25%), moderate (1-5%), or severe (<1%)
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18
Q

presentation of Hemophilia A?

A
  • easy bleeding and bruisability
  • hematomas from bleeding into soft tissues and muscles
  • hemarthroses… frequent
  • patients are at significantly increased risk for bleeding during and after surgery
19
Q

Hemophilia B

A

decrease in factor 9

  • looks like hemophilia A
  • tx with factor 9
20
Q

characteristics of Deficiency of Vit K dependent factors

A
  • bleeding/hemorrhage
  • prolonged PT
  • Deficiency of factors 2,7,9,10 ptn C and S
21
Q

Hereditary Hemorrhagic Telangiectasia

A

aka: Osler-Weber-Rendu syndrome
- the only endothelial syndrome associated with hemostatic complications
- caused by thinning of vessel walls with telangiectatic formations, arteriovenous malformations, and aneurysmal dilatations throughout the body

22
Q

Hereditary hemorrhagic telangiectasia genetics

A
  • Autosomal dominant inheritance
  • Defect in gene coding for endoglin (CD105) a membrane glycoprotein strongly expressed on endothelial cells
  • located on chromosome 9
23
Q

clinical features of hereditary hemorrhagiv telangiectasia

A
  • telangiectasias: gradually appear throughout life located in skin, mucous membranes, and visceral tissues
  • Bleeding: to mild or inapparent trauma; epistaxis is the most frequent symptom (80%)
24
Q

Course and tx of HHT?

A
  • usually benign , recurrent bleeds frequent but death from exanguination is rare
  • surgery and laser photoblation of telangiectasias of value but care must be used in selecting site
25
Q

Thrombotic disorders

A
  • antithrombin III deficiency
  • Ptn C and S deficiency
  • Factor V leiden syndrome
  • Prothrombin 20210
  • Antiphospholipid Syndrome
  • Superficial venous thrombosis
26
Q

ATIII deficiency presentation?

A
  • recurrent lower extremity thrombophlebitis and DVT, venous insufficiency, and chronic leg ulcers
  • significantly incrased risk for DVT in pregnancy i
  • diagnosis: demonstration of diminished levels of ATIII in serum (<50% of normal activity
27
Q

normal PT

A

10-15 sec

28
Q

What organ produces clotting factors

A

the liver

  • so a cirrhotic patient will have a coagulopathy
  • give them fresh frozen plasma because that has clotting factors in it!
29
Q

Fondaparinux

A

its a direct thrombin inhibitor

-used to minimize risk of post op DVT

30
Q

Where do Ptn C and Ptn S work?

A

factor 5

31
Q

Tx of ATIII deficiency

A

prophylactic tx with anticoagulants

  • pts with DVT need heparin
  • ATIII replacement therapy for ppl who dont respond to heparin
32
Q

What does Ptn C do

A

inactivates factors V and VIII

33
Q

What does Ptn S do?

A

cofactor for Ptn C

-kinda supercharges it and makes it work better

34
Q

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

A

initiation of warfarin therapy

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

35
Q

Factor 5 Leiden

A
  • abnormality of factor V at binding site for activated Ptn C
  • risk for thromboembolism
36
Q

Tx of Factor V leiden

A
  • no prior episodes: monitor; DVT prophylaxis and risk reduction
  • Prior episodes: consider lifelong anticoagulation
37
Q

Prothrombin 20210

A
  • G-A mutation resulting in increased activity for prothrombin and inability to de-activate prothrombin
  • risk of thrombosis very high…. combined with factor V leiden may account for most cases of hereditary thrmobophilia
38
Q

Tx of prothrombin 20210

A

along the same guidelines as factor V leiden

39
Q

Antiphospholipid syndrome

A

-circulating antibodies to phospholipid….

poorly understood

40
Q

associated features of Antiphospholipid syndrome

A

thromboembolic phenomena

  • miscarriage
  • thrombocytopenia
  • cerebral ischemia and recurrent stroke
  • UBO: unidentified bright objects on MRI scan
41
Q

diagnosis of Antiphospholipid syndrome

A

3 tests:

  • a prolonged phospholipid-dependent coagulation test (PTT)
  • lack of correction in mixing studies using normal plasma
  • neutralization of inhibitor with excess phospholipid
42
Q

What is DRVVT

A

Dilute russell viper venom time: may be more specific than PTT-related test

43
Q

Tx of antiphospholipid syndrome

A
  • if no thromboembolic disease hx, no benefit for anticoagulation
  • for those with a history: lifelong anticoagulation…. don’t base on a single test! Multiple positive tests over a 3-12 month period are required to make diagnosis
  • anticoagulation during pregnancy may be accomplished by SC heparin
  • Hydroxychoroquine-
44
Q

What was happening in that case with the lady with a DVT and taking oral contraceptives?

A

her bro had Factor 5 leiden… so she was a heterozygote

-that superimposed on the factor 5 leiden risk gave her a hypercoagulable state