Disorders of primary hemostasis PT 2 (Plts) Flashcards
Reactive thrombocytosis
response to blood loss, major surgery, child birth
Myeloproliferative disorders (usually high plts)
p
c
pm
e
PV, CML, primary myelofibrosis, ET
Essential thrombocythemia
increased
plt #
large
t/h
increased megakary
plts >600,000
large plt masses/giant bizarre forms
thrombotic/hemorrhage
Thrombocytopenia
c
f
dec plt production
cong. hypoplasia
fanconis
Wiskott aldrich
immune disease in males
apoptosis small plts
dec plts
May hegglin
large plts, dec number, dohle like boidies
asymptomatic
Neonatal hypoplasia
plt count?
ages?/disease assoc
lack?
ingestion of what by mom?
dec plts
newborns w rubella
lack megakary
drugs ingest by mom
Aquired hypoplasia
plts?
i
d-e
v
f
dec plts
irradiation
drugs ethanol
virsues
folate
increased plt destruction
im
dr
ne
pos
c/s
immune TP (idiopathic)
drug induced
neonate allo/auto
post transf allo immune tp
CLL/SLE
ITP
plt count
size?
BM
PFA
deficient?
plts <20,000
large plts
BM- megakary hyperplasia
PFA inc
deficient clot retraction
Chronic ITP
ages
f
what kind of episodes?
manifests as?
20-50yr
fluncuates
bleeding episodes
manifests as early AIDS
Acute ITP
in?
hxt of?
spontaneous?
limit? PLT count?
drugs?
kids
hxt of infection/immunizations
80% spontaneous remission
limit sports/activites w contanct if plts <30,000
steroids
Drug induced immune effects
a true auto ab developes….
Hapten-linkage….
Drug ab complex…
a true auto ab develops that is not dependent on presence of drug
Hapten-linkage of drug to plts then forms ab
drug-ab complex attaches to plt
Common drug induced immune effect drugs
heparin
quininine, quindine
Heparin
suspected in pts with?
suspt in pts w 30-50% plt drop
HAT
Direct non…..
NOT associated with?
direct non immune med plt activation, NOT assoc w risk of thrombosis
HIT
what happens within 7 days of plt drop?
what complex?
type 1
happens?
rarely below?
thrombosis w in 7 days of plt drop
PF4 heparin complex
type 1: 1-3 days into treatment/rarely below 100 x 103
Type II HITTS
plt count? T
plt a…. what binds to what?
heparin induced…
venous
low 20 x 103 w thrombosis
plt aggrg, HIT ab binds to heparan
Heparin induced skin necrosis
venous gas gangrene
HIT and HITTS
only active in presence of
Coumadin
LMW Heparin
Fondaparinux - doesnt?
only acitve in prescene of heparin
if anticoag must be used:
Coumadin - can inc skin necrosis
LWM heparin - not ideal
Fondaparinux - synth portion of hepain molecule Xa inhibitor doesnt cross react w ab
Testing of HIT and HITTS
ref method: serotonin
HIPA common
ELISA most common
HIPA
test for…
pt must be off… for?
use?
d….+pt…..+d
test for hep induced ab, pt msut be off heparin for 8hr
use plt aggregometer
donor plts + pt PPP + dilutions of heparin
Neonatal alloimmune TP
norm at birth and then?
same as?
plt specific ag absent in?
most likely ag?
norm at birth and then get petechiae and purpura
same as HDN (Rh-ag)
Plt sp ag absent in mom (HPA-1A)
Neonate Auto TP
not actual…
mom has
common?
high
fetal
not actual “auto”
mom has ITP/SLE
common in pregnancy, in remission can relapse
high risk delivery
fecal scalp platelet
Post transfusion purpura
how many days post transfusion?
ab forms to?
most common?
initial exposure with?
can cross?
3-12 days post transufsion rapid onset TP
ab form to ag that recipient lacks
most common HPA-1A
inital exposure by transfusion/pregnany
can cross placenta/neonatal TP
Incidental TP Pregnancy
usually…
non immune incr…
usually slight inc in plt count in preg
known cause
non immune inc plt destruction
HELLP syndrom accronym
life?
pre
in how many births?
gestation?
hemolysis
elevated liver enzymes
Low plts
life threatening preg condidtion, preeclampsia
1-150 births
27-36 wk gestation
Pre eclampsia
pregnancy induced….
preg ind hypertension w edema/proteinuria after 20wk gestation
eclampsia
severe…which there is a
intravasc
micro
T
vaso
vasc
severe preeclampsia in which there is a seizure/coma
intravas plt activation
microvasc damage
TXA2 relase
vaso spasm
vasc lesions in organs
Lab findings of HELLP
cell morphology?
high?
TP levels?
Swollen
what kind of disturbances?
hemolysis/schistocytes
high liver enzymes AST/ALT/LDH
TP <200,000
swollen hands,ace, cerebral/visual distrubances
TTP
what kind of injury? pLT?
What is broken?
Auto….against?
H
H
N
Pro
endo cell injury, PLT thrombus
large vwf broken by ADAMTS13
auto-ab AGAINST ADAMTS13 or deficient
hemorrhage
hemolysis
neurologic problems
PROGRESSIVE renal disease
HUS
resembles
in?
bacteria?
M,T
acute?
no?
resembles TTP
young children
e coli
MAHA,TP, ACUTE renal faulure and no neurological problems
