diseases of hemostasis part 2

Question 1

DIC involves all hemostatic systems

Answer 1

-vascular intima
-platelets
-WBCs
-coag cascade
-coag control pathways
-fibrinolysis

Question 2

action of DIC

Answer 2

fibrin microthrombi form and partially block small vessels and consume PLTs, coag factors, coag control proteins, and fibrinolytic enzymes

Question 3

DIC acute

Answer 3

deficiencies of multiple hemostasis components; often fatal

Question 4

DIC chronic

Answer 4

normal or elevated clotting factor levels : liver and bone marrow compensation

Question 5

first sign in DIC

Answer 5

bleeding

Question 6

primary culprit of DIC

Answer 6

circulating thrombin

activates: PLTs, coag proteins

Question 7

monocytes can also be activated and secrete

Answer 7

tissue factor by cytokines released during inflammation

Question 8

normally in hemostasis

Answer 8

plasmin acts LOCALLY to digest the solid fibrin clot

Question 9

in DIC fibrin monomers do not polymerize and circulate in plasma as

Answer 9

soluble fibrin monomers

Question 10

PLTS get activated by

Answer 10

thrombin

-keeps coag system going

Question 11

hemorrhagic outcome of DIC is overall due to

Answer 11

-thrombin activation
-circulating plasmin
-loss of control
-TCP

Question 12

free plasmin does what in DIC

Answer 12

digest factors: V, VIII, IX, XI
triggers complement leading to hemolysis
-triggers kinin system resulting in inflammation, hypotension, and shock

Question 13

symptoms of DIC

Answer 13

thrombosis causing
-renal failure
-respiratory distress
-CNS problems
-skin, bone, and BM necrosis

Question 14

what are important findings in DIC

Answer 14

-schistocytes
-increase D-dimer

Question 15

2 categories of treatment for DIC

Answer 15

• therapies that slow clotting process
  -therapies that replace missing platelets and coag factors

Question 16

almost all procoagulant proteins are made in the

Answer 16

liver

-so in liver disease factors may be decreased

Question 17

in liver disease the first factor to show decreased activity is

Answer 17

factor 7

-PT will be prolonged because factor 7 in extrinsic

-same thing happens in vit K def

Question 18

another factor decrease that is good marker of liver disease

Answer 18

factor 5

-not vitamin K dependent so not effected by dietary deficiency

Question 19

how to distinguish liver disease from vitamin k deficiency

Answer 19

factor 5 and factor 7 assay

Question 20

in end stage liver disease fibriongen levels drop to

Answer 20

<100 which is marker of liver failure

Question 21

in case of obstructive liver disease only vitamin k dependent factors affected why ?

Answer 21

absorption of vit k requires bile salts in intestinal tract

Question 22

what APRs may be unaffected or elevated during liver disease

Answer 22

vWF, factor 8 and 13

Question 23

in alcoholic cirrhosis, alcohol toxicity can suppress

Answer 23

plt production

Question 24

lab test for liver disease

Answer 24

abnormal liver function test
abnormal plt function test
PT more focused on vit k dependent factors

Question 25

in renal disease patients have both a

Answer 25

bleeding and thrombotic tendency

Question 26

lab results renal disease

Answer 26

normal plt count prolonged bleeding time PT and APTT are normal

Question 27

suppression of PLT adhesion and aggregation in renal failure could be due to PLTs being coated with

Answer 27

guanidinosuccinic acid or phenolic compounds

Question 28

what contribute to bleeding in renal failure

Answer 28

decreased RBC mass and TCP

Question 29

treatment for renal disease

Answer 29

dialysis -- can activate plts DDVAP- increase plasma concentration of ultra multimers of vWF

Question 30

vitamin k deficiency affects what factors

Answer 30

vitamin k factors

Question 31

_______ from liver are necessary for vit k absorption

Answer 31

bile salts

Question 32

without vit k what form instead

Answer 32

PIVKAs (protein from vitamin k antagonism)

Question 33

what test prolonged in vit k deficiency

Answer 33

PT - more vit k factors PTT- normal to prolonged

Question 34

what factor is most dependent the - the first to be depleted in vit k deficiency

Answer 34

7

Question 35

when is vit k deficiency seen?

Answer 35

neonatal coumadin therapy severe liver failure impaired dietary intake or absorption

Question 36

what happens to patients on antibiotics in vit k def

Answer 36

antibiotics can decrease normal flora (normal flora produces vit k)

Question 37

antiphospholipid antibodies also called

Answer 37

nonspecific inhibitors -bind variety of protein-phospholipid complexes -includes lupus anticoag, ACL, anti-beta2-GP1 antibodies

Question 38

most APL arise in response to

Answer 38

bacterial, viral, fungal infections -antibodies will go away after 12 weeks

Question 39

where do autoimmune APL found

Answer 39

lupus RA scleroderma Sjogren syndrome

Question 40

if autoimmune APL antibodies are persist known to cause

Answer 40

arterial and venous thrombosis

Question 41

diagnosis of APL

Answer 41

unexplained venous or arterial thrombosis, TCP, or recurrent fetal loss

Question 42

testing for APL- Lupus anticoag

Answer 42

clot based assay use a reagent with reduced phospholipid concentration-- sensitive to LA two common test are DRVVT and silica-based PTT

Question 43

hypercoagulability hereditary deficiency in

Answer 43

antithrombin Protein C and S patient has tendency to form clots and keep them

Question 44

Virchow's triad for hypercoagulability (contribute to thrombosis)

Answer 44

hypercoagulability hemodynamic changes endothelial injury/ dysfunction

Question 45

plasminogen deficiency causes thrombosis why?

Answer 45

patients cannot break down a clot with no plasminogen -fibrinolysis impaired -similar to AT or PC deficiency

Question 46

plasminogen deficiency test results

Answer 46

PT and APTT normal need plasminogen activity and antigenic assay to dx

Question 47

defects of activators in plasminogen deficiency

Answer 47

patients experience lifelong venous thrombotic tendencies

Question 48

AT is what kind of inhibitor

Answer 48

serine protease

Question 49

ANTI-thrombin def occurs in

Answer 49

liver disease nephrotic syndrome prolonged heparin therapy birth control DIC

Question 50

AT has activity against

Answer 50

thrombin Xa IXa XIa XIIa

Question 51

what kind of cofactor is AT

Answer 51

heparin

Question 52

protein C deficiency

Answer 52

recurrent venous thrombosis

Question 53

testing for protein C def

Answer 53

1) chromogenic testing - if this is normal perform clot based PC assay

Question 54

what is protein S deficiency linked to

Answer 54

TIA -transient ischemic attacks and stroke -recurrent thrombosis

Question 55

testing for protein C def

Answer 55

no chromagenic assay

Question 56

therapy for PS and PC def

Answer 56

factor concentrates and lifelong coumadin therapy

Question 57

factor 5 leiden mutation

Answer 57

mutation in factor 5 gene substitutes glutamine for arginine at position 506 of the factor 5 molecule -slows of resists APC hydrolysis

Question 58

how does factor 5 leiden mutation affect coagulation

Answer 58

resistant factor 5 remains active and raises the production of thrombin leading to thrombosis

Question 59

what is testing for factor 5 leiden mutation

Answer 59

APC resistance clot-based assay

Question 60

if ratio is less than 1.8 there is APC resistance why?

Answer 60

APC downregulates the clotting process and if it can't do that then the time of PTT will be shortened because unchecked clotting going on

Question 61

alpha-2-anti-plasmin deficiency

Answer 61

autosomal recessive bleeding is seen -DX chromogenic assay

Question 62

for alpha-2-anti-plasmin deficiency it is a defect in thrombolytic pathway is actually a bleeding disorder why?

Answer 62

plasmin proceeds unchecked -too much of clot breaking down