4.9 Bleeding disorders

Question 1

coagulation tests

Answer 1

bleeding time,
prothrombin time (PT),
Partial thromboplastin time (PTT)

Question 2

PT longer than normal tells you

Answer 2

dec platelet function

Question 3

PT looks at

Answer 3

external path

Question 4

Partial thrombo-plastin time (PTT) looks at

Answer 4

intrinsic path

Question 5

genetics of hemophilia A and B

Answer 5

x -linkend

Question 6

Hemophilia A

Answer 6

def in 8 (most common)

Question 7

Hemophilia B

Answer 7

def in 9

Question 8

severe activity

Answer 8

<1% - see internal bleeding and hemoarthrosis

Question 9

moderate activity

Answer 9

1-5% - has variable course

Question 10

mild activity

Answer 10

5-40% - may not even be diagnosed

Question 11

in hemophilia PTT is

Answer 11

abnormal

Question 12

what is the most common coagulation disorder

Answer 12

von Willebrand Disease

Question 13

Von Willebrand Disease

Answer 13

dec amount of function of vWB factor - rirmber vWBF also carries and stabilizes factor 8 in the ciruculation

Question 14

von Willebrand Disease subtypes

Answer 14

1 2 and 3 each with subtypes

Question 15

Most common von Willebrand Disease subtype

Answer 15

Type 1 (60-80%)

Question 16

Type I vWB diisease

Answer 16

heterozygous quantitative reduction in the amount of vWB factor (10-45% or normal),
frequently asymptomatic and not diagnosed under normal condition

Question 17

Type 2 vWB disease

Answer 17

20-30%, qualitative defect in fn but normal levels of protein, subtypes A B M N

Question 18

Type 2 vWB disease -A

Answer 18

inability to form HMW multimers

Question 19

Type 2 vWB disease - B

Answer 19

gain of fn causing binding of platelets to HMW multimers and abnormal clearance

Question 20

Type 2 vWB disease - M

Answer 20

qualitative deficit in native protein not in the ability to form multimers

Question 21

Type 2 vWB disease - N

Answer 21

decrease ability to bind factor 8 but platelet fn is normal

Question 22

Type 2 vWB disease - N is commonly misdiagnosed as

Answer 22

hemophilia A bc of the dec activity of factor 8

Question 23

Type 3 vWB disease

Answer 23

homozygous defect so extremely low levels of vWBF, most serious bleeding with reduced 8 can have hemarthrosis as observed in hemophilia

Question 24

Platelet disorders

Answer 24

bernard-soulier syndrome,

glandzman thrombasthenia

Question 25

Bernard soulier syndrom

Answer 25

def in Gp1b the receptor for vWBF

Question 26

Glandzman Thrombasthenia

Answer 26

def in Gp2b/3a the receptor for fibrinogen and required for platelet aggregation

Question 27

DIC

Answer 27

disseminated intravascular coagultion

Question 28

DIC is

Answer 28

consumptive coagulopathy, widespread fibrin thrombi in the microcirculation using up platelets and coagultion factors, activates fibrinolytic system, fibrin split products appear in blood, not a primary disease but occurs in very sick people

Question 29

DIC becomes apparent by

Answer 29

bleeding - due to all the coagulation you eat up all your platelets

Question 30

in DIC you see fibrin split products bc

Answer 30

plasmin can eat up the small thrombi that are wide spread froming the fibrin split products

Question 31

so if you see someone that is Sick with GI bleed and fibrin split products think

Answer 31

DIC

Question 32

Infarction

Answer 32

ischemic necrosis caused by occlusion of the arterial supply or the venous drainage, 99% caused by thrombotic or embolic events

Question 33

occlusion of the arterial supply

Answer 33

wedge shape (but must be con correct plane)

Question 34

red infarcts

Answer 34

venous - red indicates that the part before the occlusion is getting too much blod --hemorragic dead tissue results

Question 35

and MI might look red due to

Answer 35

reperfusion - even though the artery was pinched off it got catherized and reprofused

Question 36

if you see many areas with occlusions think

Answer 36

left heart problems/vegetation

Question 37

factors affecting infarction

Answer 37

anatomy of vasculature, rate of occlusion, organ dependence on aerobic metabolism, oxygen content of blood

Question 38

sudden occlusion is prone to

Answer 38

infarctin

Question 39

slow occlusion

Answer 39

might have nagina but since tissue gets mildly ischemic this tells the body to brow more collateral vessesl

Question 40

Shock

Answer 40

cardiovascular collapse due to reduction in cardiac output or reduction in blood volume. Results in systemic hypotension, hypoperfusion, and hypoxia

Question 41

Three forms of schock

Answer 41

cardiogenic, hypvolumic, septic

Question 42

cardiogenic shock

Answer 42

``` pump failure, muscle damage bye infarction, cardiomyopathies, extrinsic compression, arrythmias, outflow obstruction ```

Question 43

hypovolumic shock

Answer 43

blood loss from hemorrhage, fluid loss from burns, vomiting, diarrhea

Question 44

Septic shock

Answer 44

25-75% mortality rate, usual cause is endotoxin from gram negative bacteria, but other bacteria and fungal products are also inducers and organisms don't have to be alive

Question 45

LPS and Serum will induce macrophages to produce

Answer 45

TNF IL1 and IL6

Question 46

TNF and IL1

Answer 46

``` leukocyte/endothelium activation, complement activation, fever/acute phase reactants, systemic vasodialation, lowered cardiac output, vessel damage and thrombosis, ARDS and DIC ```

Question 47

Stages of Schock

Answer 47

nonprogressive, progressive, irreversible

Question 48

nonprogressive shock

Answer 48

compensatory mechanisms maintain perfusion

Question 49

pregressive shock

Answer 49

tissue hypoporfusion and beginning of dwonward spiral

Question 50

irreversible shock

Answer 50

ischemic damage to tissues such that correction in hemodynamics cannot rescue

Question 51

nightmare of shock

Answer 51

overcompensation leading to vasoconstriction, tachycardia, reanl fluid retention__hypoxia leads to anaerobic metabolism and lactic acid production inducing vasodialtion_.. cardiac output decreases, hypoxia worsens, hypoxic heart lower output, angiotensin activated, worsened systemic hypoxia