Disorders of Primary Hemostasis (vasculature/plts) pt 1 (lect 3)

Question 1

Vascular disorders
h
e
a/d
h
s
sp

Answer 1

HHT
EDS
Allergic/drug induced
HSP
Scurvy
Senile purpura

Question 2

Hereditary Hemorragic Telangiectasia

Answer 2

vessel walls reduced to single layer of EC/fragile

Telanglectasis - dialated superficial vessles that blanche w pressure unlike petechia

Bleeding: epistaxis. GI, UG, other organs

Question 3

Ehlers-Danlos syndrome

Answer 3

indian rubberman

hypermobile joints
stretchy skin

collagen disorder

Question 4

Allergic/drug induced purpuras

Answer 4

aquired
autoimmune vascular injury or

drug caused development of ab to vessel walls

Question 5

Henoch-schonlein purpura
what type of infection?
what is deposited into tissues?
physical changes?
small % advances to?
looks like?

Answer 5

aquired

child upper resp infection

IgA deposited in tissues
rash abdm pain joint pain

small % advance to renal disease

looks like meningitis

Question 6

Scurvy
decreased….
weakened

Answer 6

Vit. C deficiency

decreased synth of collagen weakened capillary walls

Question 7

Senile purpura

Answer 7

elderly
forearms and back of hands

loss of collagen/fat/elastic fibers to support small blood vessels

Question 8

Bernard-Soulier syndrome
lack of ….
what kind of disorder?
bleeding time/PFA?
plt #?
plt aggregates?

Answer 8

Lack of GP-1B on plts

adhesion disorder/function

bleeding time/PFA: increased

plts decreased, LARGE PLTS

Plt agreggates (NORMAL WITH ALL AGENTS BUT RISTOCETIN)

Question 9

Von willibrand Disease
what kind of disorder?
synth in?
stored in?

Answer 9

Plt Adhesion disorder

VWF synth in endo cells/megakary

stored in Alpha granules of plts in plasma

Question 10

VWD Type 1 defect
dec
release?
structure?

FVIII level
APTT val
PT val
PFA val

Answer 10

most common

decreased amount of all multimers
abnormal release
structure normal

LOW/NORM FVIII
APTT Val: prolonged or normal (factor 8)
PT val: norm (doesnt involv fact 8)

PFA increased

Question 11

VWF ag

Answer 11

quantitate plasma vwf (ELISA/Chemilum..etc)

separate mult on basis of molecular size
agarose gel/crossed immunoelect

Question 12

VWF activity
RIPA
use ?
normal except with?
add what to correct?

Answer 12

ristocetin induced plt agglut

plt aggregometer
uses pt prp pts own plasma/plts

normal except w ristocetin

add Np plasma and it will correct

Question 13

ristocetin cofactor (VWF:RCO)
uses
measures ability of…

Answer 13

plt aggregometer

used pt plasma and donor plts/lyoholized plts

measures ability of pt plasma vwf to agglut donor plt in presence of ristocetin

Question 14

Type II VWF (A-B,M,N)
poss due to..

Answer 14

decrease in high MW multimers
poss due to inability to stabilize large multimers

Question 15

Type III VWF

Answer 15

most severe
all multimeres absent
reduced synth or rapid breakdown at sythesis sites

Question 16

PLT defect

Answer 16

GP-1B increases affinity VWF plt agglut and are removed

Question 17

other plt adhesion problems

Answer 17

autoimmune disorder (IgG anti-plt ab)

Myeloproliferative disorders

MM/WM (igs coat plts/collagen fibers)

Chronic liver Disease (alc)

Drugs (plt membrane mods)

Question 18

Glanzmanns thrombasthenia
what kind of disorder
plt lacks?

PFA
PT
APTT
PLT count
Plt aggreg?

Answer 18

aggregation disorder

plt lacks GP IIB/IIIA

PFA increased
PT norm
APTT norm
PLT count norm

Plt aggreg norm with ONLY RISTOCETIN

Question 19

Hereditary aibrinogenemia
uremia?
aspirin therapy - inhibits

Answer 19

very little fibrinogen (no plt to plt)
aggregation problem

Uremia: toxins interfere w plt function

Aspirin therapy: inhibits cyclooxygen needed for TXA2 prod
disorder of release

Question 20

Aspirin Resistance
detect w
what is most sensitive

Answer 20

up to 22% of pts taking aspirin become resistent

assoc w thrombosis (higher in MI/stroke)

detect w plt aggrg studies

arachidonic acid curve is most sensitive

Question 21

Storage pool diseases

Answer 21

release disorder

lack dense and alpha granules of plts
no normal release

Question 22

alpha stroage pool

Answer 22

lack dense granules
release problem

Question 23

Hermansky-Pudlack syndrome
what kind of deficiency
plts?
deficient…system
what kind of disorder?

Answer 23

dense granule deficiency
swiss cheese plts
def canicular system

release disorder

Question 24

Gray plt syndrome

Answer 24

decreased alpha plt granules
hypo/agranular plts

Question 25

Wiskott aldrich syndrome
what kind of disorder
plts?
decreased?
what dysfunction?

Answer 25

release disorder

micro plts

decrease alpha and dense granules

B/T cell dysfunction

Question 26

Chediak higashi

Answer 26

lack dense granules

lysosome in pmn looks like dohle body

release disorder