Disorders of primary hemostasis PT 2 (Plts)

Question 1

Reactive thrombocytosis

Answer 1

response to blood loss, major surgery, child birth

Question 2

Myeloproliferative disorders (usually high plts)
p
c
pm
e

Answer 2

PV, CML, primary myelofibrosis, ET

Question 3

Essential thrombocythemia
increased
plt #
large
t/h

Answer 3

increased megakary

plts >600,000

large plt masses/giant bizarre forms

thrombotic/hemorrhage

Question 4

Thrombocytopenia
c
f

Answer 4

dec plt production

cong. hypoplasia
fanconis

Question 5

Wiskott aldrich

Answer 5

immune disease in males
apoptosis small plts
dec plts

Question 6

May hegglin

Answer 6

large plts, dec number, dohle like boidies
asymptomatic

Question 7

Neonatal hypoplasia
plt count?
ages?/disease assoc
lack?
ingestion of what by mom?

Answer 7

dec plts
newborns w rubella

lack megakary
drugs ingest by mom

Question 8

Aquired hypoplasia
plts?
i
d-e
v
f

Answer 8

dec plts
irradiation
drugs ethanol
virsues
folate

Question 9

increased plt destruction
im
dr
ne
pos
c/s

Answer 9

immune TP (idiopathic)
drug induced
neonate allo/auto
post transf allo immune tp
CLL/SLE

Question 10

ITP
plt count
size?

BM

PFA
deficient?

Answer 10

plts <20,000
large plts

BM- megakary hyperplasia

PFA inc

deficient clot retraction

Question 11

Chronic ITP
ages
f
what kind of episodes?
manifests as?

Answer 11

20-50yr
fluncuates
bleeding episodes
manifests as early AIDS

Question 12

Acute ITP
in?
hxt of?
spontaneous?

limit? PLT count?

drugs?

Answer 12

kids
hxt of infection/immunizations
80% spontaneous remission

limit sports/activites w contanct if plts <30,000

steroids

Question 13

Drug induced immune effects
a true auto ab developes….

Hapten-linkage….

Drug ab complex…

Answer 13

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

Question 14

Common drug induced immune effect drugs

Answer 14

heparin
quininine, quindine

Question 15

Heparin
suspected in pts with?

Answer 15

suspt in pts w 30-50% plt drop

Question 16

HAT
Direct non…..

NOT associated with?

Answer 16

direct non immune med plt activation, NOT assoc w risk of thrombosis

Question 17

HIT
what happens within 7 days of plt drop?
what complex?

type 1
happens?
rarely below?

Answer 17

thrombosis w in 7 days of plt drop
PF4 heparin complex

type 1: 1-3 days into treatment/rarely below 100 x 103

Question 18

Type II HITTS
plt count? T
plt a…. what binds to what?

heparin induced…
venous

Answer 18

low 20 x 103 w thrombosis

plt aggrg, HIT ab binds to heparan

Heparin induced skin necrosis

venous gas gangrene

Question 19

HIT and HITTS
only active in presence of

Coumadin
LMW Heparin
Fondaparinux - doesnt?

Answer 19

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

Question 20

Testing of HIT and HITTS

Answer 20

ref method: serotonin
HIPA common
ELISA most common

Question 21

HIPA
test for…
pt must be off… for?

use?

d….+pt…..+d

Answer 21

test for hep induced ab, pt msut be off heparin for 8hr

use plt aggregometer

donor plts + pt PPP + dilutions of heparin

Question 22

Neonatal alloimmune TP
norm at birth and then?

same as?

plt specific ag absent in?
most likely ag?

Answer 22

norm at birth and then get petechiae and purpura

same as HDN (Rh-ag)

Plt sp ag absent in mom (HPA-1A)

Question 23

Neonate Auto TP
not actual…
mom has

common?

high
fetal

Answer 23

not actual “auto”
mom has ITP/SLE

common in pregnancy, in remission can relapse

high risk delivery
fecal scalp platelet

Question 24

Post transfusion purpura
how many days post transfusion?

ab forms to?

most common?

initial exposure with?
can cross?

Answer 24

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

Question 25

Incidental TP Pregnancy
usually…
non immune incr…

Answer 25

usually slight inc in plt count in preg
known cause

non immune inc plt destruction

Question 26

HELLP syndrom accronym

life?
pre

in how many births?
gestation?

Answer 26

hemolysis
elevated liver enzymes
Low plts

life threatening preg condidtion, preeclampsia

1-150 births
27-36 wk gestation

Question 27

Pre eclampsia
pregnancy induced….

Answer 27

preg ind hypertension w edema/proteinuria after 20wk gestation

Question 28

eclampsia
severe…which there is a

intravasc
micro
T
vaso
vasc

Answer 28

severe preeclampsia in which there is a seizure/coma

intravas plt activation
microvasc damage
TXA2 relase
vaso spasm
vasc lesions in organs

Question 29

Lab findings of HELLP
cell morphology?
high?
TP levels?
Swollen
what kind of disturbances?

Answer 29

hemolysis/schistocytes

high liver enzymes AST/ALT/LDH

TP <200,000

swollen hands,ace, cerebral/visual distrubances

Question 30

TTP
what kind of injury? pLT?
What is broken?

Auto….against?

H
H
N
Pro

Answer 30

endo cell injury, PLT thrombus

large vwf broken by ADAMTS13

auto-ab AGAINST ADAMTS13 or deficient

hemorrhage
hemolysis
neurologic problems
PROGRESSIVE renal disease

Question 31

HUS
resembles
in?
bacteria?
M,T
acute?
no?

Answer 31

resembles TTP

young children

e coli

MAHA,TP, ACUTE renal faulure and no neurological problems