Disorders of Primary Hemostasis (vasculature/plts) pt 1 (lect 3) Flashcards
Vascular disorders
h
e
a/d
h
s
sp
HHT
EDS
Allergic/drug induced
HSP
Scurvy
Senile purpura
Hereditary Hemorragic Telangiectasia
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
Ehlers-Danlos syndrome
indian rubberman
hypermobile joints
stretchy skin
collagen disorder
Allergic/drug induced purpuras
aquired
autoimmune vascular injury or
drug caused development of ab to vessel walls
Henoch-schonlein purpura
what type of infection?
what is deposited into tissues?
physical changes?
small % advances to?
looks like?
aquired
child upper resp infection
IgA deposited in tissues
rash abdm pain joint pain
small % advance to renal disease
looks like meningitis
Scurvy
decreased….
weakened
Vit. C deficiency
decreased synth of collagen weakened capillary walls
Senile purpura
elderly
forearms and back of hands
loss of collagen/fat/elastic fibers to support small blood vessels
Bernard-Soulier syndrome
lack of ….
what kind of disorder?
bleeding time/PFA?
plt #?
plt aggregates?
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)
Von willibrand Disease
what kind of disorder?
synth in?
stored in?
Plt Adhesion disorder
VWF synth in endo cells/megakary
stored in Alpha granules of plts in plasma
VWD Type 1 defect
dec
release?
structure?
FVIII level
APTT val
PT val
PFA val
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
VWF ag
quantitate plasma vwf (ELISA/Chemilum..etc)
separate mult on basis of molecular size
agarose gel/crossed immunoelect
VWF activity
RIPA
use ?
normal except with?
add what to correct?
ristocetin induced plt agglut
plt aggregometer
uses pt prp pts own plasma/plts
normal except w ristocetin
add Np plasma and it will correct
ristocetin cofactor (VWF:RCO)
uses
measures ability of…
plt aggregometer
used pt plasma and donor plts/lyoholized plts
measures ability of pt plasma vwf to agglut donor plt in presence of ristocetin
Type II VWF (A-B,M,N)
poss due to..
decrease in high MW multimers
poss due to inability to stabilize large multimers
Type III VWF
most severe
all multimeres absent
reduced synth or rapid breakdown at sythesis sites
PLT defect
GP-1B increases affinity VWF plt agglut and are removed
other plt adhesion problems
autoimmune disorder (IgG anti-plt ab)
Myeloproliferative disorders
MM/WM (igs coat plts/collagen fibers)
Chronic liver Disease (alc)
Drugs (plt membrane mods)
Glanzmanns thrombasthenia
what kind of disorder
plt lacks?
PFA
PT
APTT
PLT count
Plt aggreg?
aggregation disorder
plt lacks GP IIB/IIIA
PFA increased
PT norm
APTT norm
PLT count norm
Plt aggreg norm with ONLY RISTOCETIN
Hereditary aibrinogenemia
uremia?
aspirin therapy - inhibits
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
Aspirin Resistance
detect w
what is most sensitive
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
Storage pool diseases
release disorder
lack dense and alpha granules of plts
no normal release
alpha stroage pool
lack dense granules
release problem
Hermansky-Pudlack syndrome
what kind of deficiency
plts?
deficient…system
what kind of disorder?
dense granule deficiency
swiss cheese plts
def canicular system
release disorder
Gray plt syndrome
decreased alpha plt granules
hypo/agranular plts
Wiskott aldrich syndrome
what kind of disorder
plts?
decreased?
what dysfunction?
release disorder
micro plts
decrease alpha and dense granules
B/T cell dysfunction
Chediak higashi
lack dense granules
lysosome in pmn looks like dohle body
release disorder
