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Haem/Onc > Platelet Disorders, ASPHO > Flashcards

Platelet Disorders, ASPHO Flashcards

1
Q

plt lifespan?

A

7-9 days

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2
Q

how do plts get removed from circ?

A

monocyte-macropahge system

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3
Q

sites of destruction/utilization of plts?

A

spleen, liver, endothelial cell junction

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4
Q

% of circ’ing plts in spleen?

A

25-35%

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5
Q

causes of low plts?

A
  • shortened life span (immune or not)
  • plt seq
  • plt loss or dilution (massive transfusion)
  • decrased production (marrow issue, folate def, b12 def)
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6
Q

DDX for low plts in newbron?

A
  • impaired production (placental insuff, fetal hypox, fetal iugr, perinatal drugs)
  • consumption/sequestration (nec, RDS, thrombosis, hemangiomas, splenic seq)
  • infectoius (TORCH, sepsis)
  • immune destruction
  • genetic (BMF, trisomy 18/13/21, IEM)
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7
Q

Neonatal alloimmune thrombocytopenia: dx how?

A
  • anti-plts alloAbs in teh mom

- document fetomat incompat

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8
Q

NAIT antigens involved in caucasians?

A

HPA1>, 5, 3

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9
Q

NAIT antigens in asian population?

A

HPA-4 and 5

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10
Q

NAIT managemetn?

A
  • daily plt count
  • HUS for ICH
  • tx if plts <30k
  • transfuse maternal plts
  • if can’t get mat plts, give HPA1a neg plts
  • or donor plts +/- IVIG and corticosteroids
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11
Q

moms with prior hx of NAIT..manage how?

A

future pregs more severe: maternal IVIG weekly +/- daily corticosteroids

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12
Q

maternal ITP __ occur while mom in remmission

A

CAN

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13
Q

tx for matenral ITP?

A

IVIG 1 g/kg for 1-2 dose

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14
Q

maternal ITP prog?

A

nadir aroudn 3-4 days of age…most infants show count recovery by 7 days

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15
Q

what’s more common: maternal ITP ro NAIT?

A

NAIT

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16
Q

describe antigens/abs in maternal ITP vs NAIT

A

maternal ITP: antigen present on mom AND baby’s plts…NAIT: HPA-1a antigen on baby’s plts while mom is HPA-1a neg

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17
Q

does ab affect plt functionin maternal itp? nait?

A

itp: no
nait: YES, via GP2b/3a

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18
Q

waht’s more severe- mat itp or nait?

A

nait! can get ICH, rarely fatal

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19
Q

maternal itp, nait tx?

A

itp: observation, IVIG for clinical bleeding
nait: maternal plts, HPA-1 neg plts, IVIG+ random donor…goal >30-50

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20
Q

peak age itp?

A

toddlers, adolescents

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21
Q

ITP: natural hx = rise in plts coutn within how long?

A

1-3 weeks

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22
Q

prog ITP?

A

80% resolve within 12 months

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23
Q

red flags in ITP for other dx?

A

-anemia
-abnormal MCV
-low ANC
hepatosplenoemgaly
-constitutional sx

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24
Q

ITP tx?

A
  • observation (most)
  • corticosteroids
  • IVIG
  • ANti-D= winrho
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25
Q

AE of winrho?

A

hemolysis…only works in pts who are Rh+ adn have spleen in tact

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26
Q

2nd line tx in ITP?

A
  • splenectony
  • ritux
  • eltrombopag
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27
Q

risks of splenectomy? resposne rate?

A
  • sepsis, thrombosis

- 60-80%

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28
Q

risks of ritux? resposne rate for ITP?

A

infusion rxn, serum sickness, CVID, 30%

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29
Q

risks fo TPO agonsists?

A

thrombocytosis, bone develop of marrow reticulin, thormbosis

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30
Q

non-immune low plts ddx, other than marrow issue?

A
  • DIC
  • TTp/hus
  • cardiopulm bypass
  • sepsis
  • preeclampsia/HELPP
  • catastrophic antiphospholipid antibides
  • transplant related
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31
Q

TTP smear findings?

A

low plts
large pts
schistocytes

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32
Q

HUS pathophys?

A

endothelial activation
leuk activation
plt activation

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33
Q

HUS associatd iwth what trigger?

A

e. coli verotoxin O157:H7 and strep pneumonia

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34
Q

HUS features?

A

MAHA, renal failure

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35
Q

tx for HUS?

A

dialysis and other supportive care; no need for plasmapheresis

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36
Q

atypical HUS difference?

A

no prodrome illness..an have muts in or abs to factor H, I or membrane co-factor protein…can give eculizumab

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37
Q

ttp: pathophys

A

def or abs to ADAMTS13, which normally breaks down very large vWF multimers–> large vWF multimers–> microthrombi

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38
Q

pentad for ttp?

A 
MAHA
thrombocytopenia
neuro findings
renal manifestations
fever
...must have no other identificable cause
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39
Q

dx features of ttp?

A 
schistoytse, helmet cells
elevated LDH
elevated retics
neg coombs
normal PT, PTT, fibrinogen, etc
renal manfisations not severe
reduced ADAMTS13
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40
Q

ttp mort rate if no therpay?

A

80%! start plasmapheresis right away!

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41
Q

ttp tx?

A

daily plasma exchanrge until control of hemolytic anemia and normal plt count, then altenratve day and/or less frequently…start corticosteroids

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42
Q

relapse rate in ttp?

A

high, at 20-30%

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43
Q

congen ttp: maange how?

A

infusions of ffp q2-3 weeks to maintain ADAMTS13>5%

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44
Q

evan’s syndrome smear: see large plts adn? other lab findings? tx?

A

spherocytes…DAT+…corticosteroids

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45
Q

give 4 inherited d/os of plt function

A

glanzmann thrombashenia
bernard soulier
grey plt syndrome
hermansky-pudlak syndrome

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46
Q

alpha grandules contain what?

A
  • VWF
  • fibrinogen
  • PF4=neutralizes heparin-like molecules
  • Growth factors: PDGF, TGF-beta1
  • coag factors (factor V)
  • p-selectin= cell adhesion molec for plt aggreg
47
Q

dense granules contain?

A
  • ADP/ATP, activates and recruits plts
  • serotonin, vasocontrictor
  • calcium: needed for fibrinogen binding to GP 2b3a
48
Q

which granules require EM to see?

A

DENSE

49
Q

steps that plts undergo?

A

adhesion-> activation-> aggregation–> propogration of coagulation

50
Q

what happens during plt adhesion?

A

plts bind to subendothelial vWF/collagen via the plt GPIb-IX-V adn GPVI

51
Q

what happens during plt activation?

A

plt shape change exposes the GPIIb/IIIa receptors

52
Q

what happens during plt aggreagtion?

A

cross-linking of plt activated GP2b/3a by fibrinogen or vWF

53
Q

what happens during plt propogation of coagulation?

A

activated plts provide an anionic aminophospholipid (PL) rich surface for the assemby of procoagulant enzyme complexes

54
Q

macrothrombocytoepnia seen in what 2 disorders?

A

bernard-soulier

gray plt syndrome

55
Q

normal plt count seen in what 2 plt disorders?

A

glanzmann

hermansky-pudlak

56
Q

describe pfa-100

A

exposure of blood to a membrane coated with collagen and adenosine diphosphate or collagen adn epi–> foramtion fo plt plug closes aperture

57
Q

in aspirin use, what resul will you see in pfa-100?

A

prolonged col/epi results, normal col/adp result

58
Q

4 agonsits in plt aggreg

A 
col
arachidonic acid
epi
ADP
ristocetin
59
Q

plt aggreg measures?

A

light transmission thru plt-rich plasma or whole blood

60
Q

limitations of plt aggreg?

A

not reliable when pl count <100

not clear how to intrepret mild defects

61
Q

arachidonic acid agonist checks what in plt aggreg?

A

gets converted to thromboxane a2 by cyclooxygenase

62
Q

genes in glanzmann

A

ITGA2B, ITGB3

63
Q

Bernard-soulier: inheritance?

A

AR

64
Q

BSS: abnormal or absent surface receptor for VWF: waht is it?

A

GP Ib/IX…CD42

65
Q

gnes in BSS?

A

GP1BA, GP1BB, GP9

66
Q

dx BSS how?

A

plt aggreg
flow
macothrombocytoepnia

67
Q

tx for GT, BSS

A

supportive
plt transfusion (risk abs)
rF7a
sct

68
Q

alpha granule: disorder where they’re absent?

A

gray plt syndrome

69
Q

2 disordres wehre there’s an absence in dense granules?

A

hermansky-pudlak syndrome

chediak-higashi syndrome

70
Q

grey plt syndrome inheritance?

A

AR, AD

71
Q

gene in grey plts?

A

NBEAL2

72
Q

confirm grey plts how after seeing on light micrsocopy?

A

EM

73
Q

grey plts associated with waht 2 things?

A

myelofibrosis, splenomegaly

74
Q

tx for grey plts?

A

transfusion
desmopressin
splenectomy

75
Q

HPS: 2 clinical features?

A

mild bleeding
occulocuaneous albinism
nystagmus

76
Q

HPS: inherit?genes?

A

AR…HPS1, HPS3, HPS5, etc

77
Q

HPS tx?

A

plt transfusions

78
Q

see waht on aggregatometry for HPS?

A

absent ATP secretion adn secondary wave of aggregation with ADP and epi…line will go back upwards again for ADP

79
Q

plt count in HPS?

A

normal

80
Q

Wiskott-Aldrich syndrome: inheritance? gene?

A

x-linked; WASP

81
Q

WASP gene encodes what?

A

surface glycoprotein CD43, an actin-binding signaling protein

82
Q

features of WAS

A
  • low plts
  • small plts
  • defects in T cell adn B-cells
83
Q

x-linked thrombocytopenia gene?

A

also WASP…microthrombocytoepnia but no eczema or immunodef

84
Q

traid in WAS

A

low plts
eczema
frequent infections

85
Q

dx WAS how? 2

A

gene testing

flow for WASP protein

86
Q

complications of WAS?

A

hemorrahge
infection
autoimmune disordrs
malig like ALL, lymphoma

87
Q

tx of WAS (3)

A

supportive for eczema, infection
splenectomy
SCT

88
Q

MYH9-related disorders: inheritance?

A

AD

89
Q

MYH9 dx?

A

leukocyte inclusions= dohle bodies

gene testing

90
Q

features of MYH9 related disordres? 3

A

renal failure
SNHL
cataracts

91
Q

MYH9 management

A

supportive

92
Q

TAR = thrombocytopnia absent radii syndrome…thumbs are?

A

normal

93
Q

heme issue in TAR?

A

absent megakaryocytes with elevated TPO levels

94
Q

TAR tx?

A

plt transfusions…most have increase in plt count by 12-24 months though…self-correction!

95
Q

cleft palate, heart defect, learning issue, low plts…think?

A

velocardiofacial syndrome

96
Q

congential heart disease, dev delay, faical dysmorph, short stature, low plts…think?

A

jacobson syndrome/paris-trousseau

97
Q

size of plts in MYH9?

A

large

98
Q

MYH9 gene?

A

MYH9

99
Q

other than absent radii and low plts, 2 other features of TAR?

A

cow milk intolerance
cardiac issues
renal issues

100
Q

organ dysfunction–> plt dysfunction? 1 reason?

A

renal; decreased thromboxane A2 formation

101
Q

ASA leads to irreversible inhibition of what? last for how long?

A

cyclooxygenase 1; 7 days

102
Q

NSAID effect on plts: reversible or irrev? of what specifically?

A

reversible; cyclooxygenase 1

103
Q

how does cyanotic heart disease and ECMO affect plts?

A

plt activation

104
Q

other than NSAIDs adn ASA, other drugs–> plt dysfunction?

A

valporic acid
psychotropic drugs
semi synthetic penicillin derviatives

105
Q

what does plt aggreg look like on ASA?

A

ADP goes down a bit then BACK UP, everythign else is really dampened

106
Q

mange acquire plt dysfucntion how?

A 
remove offending agent
tx underlying disease
lcoal pressures, pressure
TXA
desmopressin
conjguated estrogens
plt tranfusions
rbcs if anemic
107
Q

mut in essential thrombocytosis?

A

JAK>CALR, MPL

108
Q

causes of reactive thrombocytosis

A 
inflamm
iron def
marrow recovery
asplenia
young infants (esp prem)
109
Q

reactive thrombocytosis def’n

A

> 450k (usually caps at 1 million)

110
Q

for seconary thrombocytosis, do they get clots?

A

no

111
Q

ET: clinical featuers?

A

splenomegaly
bleeding-acquired vWD
-thrombosis
-numbness and tingling, fatigue, headache, vision changes

112
Q

ET plt count?

A

> 450k

113
Q

ET lab test?

A

high levels of thrombopoeitin

114
Q

management of ET?

A

ASA
HU
anagrelide

Haem/Onc (30 decks)

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