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Erez's HEM TEST notes 2024 > Thrombosis facts > Flashcards

Thrombosis facts Flashcards

1
Q

Rovelizumab vs Eculizumab in PNH

A

Same effectiveness
Revu given once every 8 weeks vs 2 weeks with eco

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2
Q

How to manage breakthrough hemolysis in PNH

A

-Shorten time interval of Eco to 10 days
- Switch to Revu
- Switch to C3 inhibitor

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3
Q

Which C5 inhibitor is approved in pregnancy

A

Ecu
Does not cross placenta in high concentration

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4
Q

Anticoagulant effect on LAC

A

DOACs cause false positive

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5
Q

Definition of TTP excerbation

A

PLT < 150
30 days after cessation of PEX or caplacizumab

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6
Q

Predictors of TTP exacerbation/relapse

A

low ADAMTS13
high Ab titers
black race

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7
Q

Only treatment shown to reduce exacerbation in TTP

A

Caplacizumab

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8
Q

Exacerbation rate after treatment with PEX in TTP

A

20-30%

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9
Q

Prednisone tapering in TTP

A

Quickly over 4 weeks

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10
Q

Caplacizumab Tx in TTP

A

HERCULES
Phase III
vs placebo
Primary end point - PEX days, PLT recovery
Improves everything (composite of mortality, thrombosis, recurrence)
Given for 1 month with option to prolong to 2 months

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11
Q

Caplacizumab duration of Tx

A

4 weeks
Can prolong to 8 weeks if ADAMTS13 still < 10%

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12
Q

Interval of FU in TTP

A

1/w for a month
1/m for 3-6 months
1/ 3-6 months

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13
Q

Threshold for preemptive Tx in TTP

A

ADAMTS13 < 10%

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13
Q

Other non rituximab Tx in TTP

A

CSA- mildly effective, prolonged Tx needed
Splenectomy (not during acute exacerbation)- 70-80% ORR

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14
Q

Neurocognitive late effects of TTP

A

Neurocognitive impairment- 60%
Mild depression- 80%
MDD- 20%

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15
Q

DOACs in cancer associated VTE

A

Less thrombosis with more bleeding

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16
Q

Thromboprophylaxis with DOACs in cancer

A

Apixaban- positive study 4% vs 10%
Rivaroxaban- negative study

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17
Q

APLS M:F

A

1:5

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18
Q

CAPS mortality

A

30% with AC, Steroids and PEX

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19
Q

Recurrent thrombosis % in APLS pts on VKA

A

2-5%

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20
Q

Options for VKA failure in APLS

A

Close INR monitoring (if non-compliance)

#Increase INR target to 3-4
#LMWH at 125%
# Fondaparinux
# add Asp/plaquenil/Statin/ Vitamin D/ immunosuppressive Tx/ Vasodilators

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21
Q

Management of microvascular complications in APLS

A

Aspirin
Immunosuppressive Tx
Complement inhibition

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22
Q

ESRD in aHUS

A

66%
Probably better with current Tx

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23
Q

PLASMIC score

A

PLT < 30
Hemolysis
No active cancer
No Hx of solid trasplant
MCV< 90
INR < 1.5
Cre < 2

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24
Q

Tx of pregnant aHUS pts

A

Eculizumab
need to increase dose

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25
Q

Managment of anti-C5 Tx in aHUS pts with infection/surgery

A

Continue Tx with anti-C5 and ABx

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26
Q

Tx of choice in acute aHUS

A

Eculizumab
Preferred over ravulizumab
Better when diagnosis not certain
Faster renal recovery

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27
Q

Median time to PLT recovery in aHUS

A

10 days

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28
Q

Monitoring response to Tx in aHUS

A

Total complement activity [CH50] < 20%) after 7 days- not possible with ravulizumab
as well as LDH, CBC normalization and Cre improvemet

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29
Q

Risk of relapse after anti-c5 discontiuation in aHUS

A

30-60%
Higher in CFH

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30
Q

Prophylactic ABx in aHUS

A

Penicillin during c5 inhibition
Vaccination is important but not enough

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31
Q

Paget Schroetter syndrome

A

Effort-induced thrombosis of the axillary and subclavian veins
compression of the subclavian vein at the thoracic outlet

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32
Q

APL ab presence

A

50% pts with SLE
5% of normal population

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33
Q

Difficulty with heparin Tx in APLS

A

aPTT may be elvated due to LAC
monitor through anti-Xa

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34
Q

Dosing of UFH

A

80 U/kg bolus
18 U/kg/h maintenence
Goal aPTT of x1.5-2

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35
Q

VWD type 2A

A

Absence of intermediate and high molecular weight multimers

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36
Q

VWD type 1C

A

increased clearence of VWF
Resembling type 1
but short response to DDAVP

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37
Q

VWD type 2B

A

Increased binding to PLT
thrombocytopenia
DDAVP is contraindicated bcs can worsen thrombocytopenia

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38
Q

VWD type 2M

A

Decreased affinty to PLT

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39
Q

VWD type 2N

A

Reduced binding to FVIII
FVIII very low
only type 2 that has high VWF Act/Ag (>0.7)
“NEIGHT”
“I 8 a sNake”

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40
Q

Limitations for VWF testing

A

VWF and FVIII are acute phase reactants

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41
Q

Significance of low dose ristocitin assay

A

Will show platelet aggregation in VWD type 2B

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42
Q

Diagnosis of VWD type 1

A

VWF Ag and Act <30
or <50 if bleeding symptoms
Increase after DDAVP
Act/ag >0.7

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43
Q

How to discriminate between VWD type 1 and type 1C

A

in 1C there is a decrease in VWF 4 hours post desmopressin
in 1C VWFpp/VWF >3
VWFpp is the propeptide of VWF and in 1C is not effected by the fast breakup of VWF

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44
Q

VWD 2B diagnosis

A

Low dose ristocitin causes agglutination in concentrations not usually able to cause activation

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45
Q

VWD 2B Tx

A

VWF

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46
Q

Platelet type VWD

A

Mutation in PLT causing enhanced binding to VWF
Tx with PLT
VWF is not effective because VWF will not be able to activate PLTs

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47
Q

VWD type 2N diagnosis

A

VWF:FVIII binding assays
May be falsely classified as mild hemophilia A
AR inhertiance or no response to FVIII Tx should raise the suspicion

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48
Q

Heide syndrome VWD type

A

2A
Loss of HMWM

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49
Q

Hemophilia severity

A

Mild < 40%
Moderate < 5%
Severe <1%

50
Q

aPTT measurement method

A

Clot formation after incubaiton with phospholipid, calcium, and a contact activator

51
Q

Effect of emicizumab on PTT

A

Shortens it substantially

52
Q

FVIII concentrate dosing

A

1U/kg increases 2% activity
50U/kg is expected to raise levels to 100%

53
Q

FIX concentrate dosing

A

1U/kg increases 1% activity
100U/kg is expected to raise levels to 100%

54
Q

Laboratory goal of prophylaxis in hemophilia

A

Factor > 1%

55
Q

Tx of choice for hemophilia pts with ab and breakthrough bleeding on emicizumab

A

rFVIIa

56
Q

Clinical criteria of APLS

A

ATE
VTE
microvascular- livedo, nephropathy, PAH, adrenal hemorrhage, MI
Cardiac valve
Obstetric
Thrombocytopenia

57
Q

New APLS criteria

A

EULAR 2023
3 points clinical
3 points labratory

58
Q

When to stop anti-PLT before surgery

A

Prasugrel 7 days
Clopidogrel, Ticagrelor- 5 days
Usually no need for bridging

59
Q

When to stop AC before surgery

A

High risk- 2 days
Low risk- 1 day
Except Dabigatran with CrCL<50 - 4 days

60
Q

Kasabach-Merritt syndrome

A

Large hemangioma causing DIC

61
Q

Which DOAC is a prodrug

A

Dabigatran

62
Q

DOAC vs LMWH in cancer

A

Lower VTE with higher bleeding rate
Especially GI
Apixaban seems safest

63
Q

VTE prophylaxis in ambulatory cancer pts

A

KHORANA score
When receiving chemo even if ambulatory- reduces mortality
commonly used only in high risk pts

64
Q

Factors in Khorana score

A

Type of cancer
CBC
Obesity

65
Q

DOAC as prophylaxis in cancer pts

A

Lower VTE with higher bleeding rate
Apixaban- AVERT
Rivaro- CASSINI

66
Q

VTE in pregnancy incidence

A

1-2/100K

67
Q

Coagulation factors that is decreased in pregnancy

A

PS

68
Q

Which thrombophilia pts need prophylaxis during pregnancy

A

Homozygotes FVL/PT
Combined thrombophilias
PC/PS/AT with FHx

69
Q

Which thrombophilia pts without FHx need prophylaxis postpartum

A

APLS- laboratory
AT
Homozygotes

70
Q

Tx of pregnant women with laboratory APLS

A

Aspirin ante partum
LMWH postpartum

71
Q

Tx of pregnant women with previous VTE

A

HIGHLOW study
low dose LMWH as efficient as intermediate dose

72
Q

Risk of VTE in pregnancy for pts with previous estrogen provoked VTE

A

Ante partum >5%
post partum >10%

73
Q

Prevalence of gestational thrombocytopenia

A

~5%

74
Q

2nd line options in ITP during pregnancy

A

Azathioprine
Splenectomy
Anti-D?

75
Q

Tx of TTP during pregnancy

A

PEX + Steroids

76
Q

Contraindications for HRT

A

Smoker above 35 YO
Previous ATE
DM with complications
Uncontrolled HTN
Cirrhosis
Breast Ca
Migraine with neuro S/Sx

77
Q

% of pts diagnosed with thrombophilia after pregnancy VTE

A

~50%

78
Q

Conditions associated with
reduced VWF

A

Hypothyroidism
Blood type O

79
Q

VWD types that are AR

A

2N
3

“N”ot to marry your sister (-;

80
Q

Hemophilia A: Hemophilia B prevalence

A

Hemophilia A more prevelant
5:1

81
Q

Titer of hemophilia Ab significance in Hemophilia A pts

A

> 5 BU- high
<5 BU- low
in low titers factor replacement still possible

82
Q

Glanzmann thrombasthenia platelet aggregation

A

Lack of response to agonists except ristocetin

83
Q

Bernard-Soulier syndrome platelet aggregation

A

Lack of response to ristocetin with a response to all other agonists

84
Q

Congenital bleeding diseases with normal coagulation tests

A

FXIII
Dysfibrinogenemia
Functional platelet defects
Collagen disorders

85
Q

Worst mutation in aHUS

A

Factor H, Factor B- consider continuous C5 inhibition

86
Q

CAPS incidence

A

1% of APLS
2:1 F:M
50% as first APLS diagnosis
~50% moratlity

87
Q

Tx of CAPS

A

AC + Steroids +- IVIG/PEX (depending on severity)

88
Q

Tx of ESUS

A

Aspirin
Rivaroxaban not more effective and more bleeding

89
Q

Factor XI inhibitor in AF

A

Trial terminated early due to higher thromobosis compared to apixaban

90
Q

Aspirin vs LMWH as prophylaxis after fractures

A

Same efficacy
Dose was bid (for both) which I don’t understand

91
Q

Efanesoctocog alfa

A

Novel Tx of severe hemophilia A
inhibits VWF clearance of FVIII
allowing FVIII a longer half life
reduces bleeding event rates

92
Q

Factor XI inhibitor in post TKR surgery thromboprophylaxis

A

Same efficacy with less bleeding

93
Q

C3 inhibition in PNH pts

A

90% ORR
for pts failing C5 inhibition
And also 1st line

94
Q

Cocizumab

A

TFPI inhibitor
Effective in hemophilia A and B with inhibitors
vs no prophylaxis
reduced bleeding

95
Q

Tranxemic acid in prehospital management of trauma pts

A

No functional benefit of pre + post
hospital administration

96
Q

Tranexamic acid pre surgery

A

Reduces bleeding

97
Q

Gray PLT syndrome

A

Low/absent of alpha granules
Progressive thrombocytopenia with large hypogranular PLT
PMF

98
Q

Chediak Higashi syndrome

A

Infections
Albinism
Giant granules in neutrophils
Thrombocytopathy
Neuropathy

99
Q

May-Hegglin anomaly

A

Macrothrombocytopenia
Dohle bodies in granulocytes
Hearing loss
Cataract
Renal failure

100
Q

Wiskott-Aldrich syndrome

A

X linked

Immunodeficiency
Thrombocytopenia- low MPV
Eczema

101
Q

FXI inhibtor after knee surgery

A

As effective as LMWH

102
Q

Effect of aging on vWF

A

vWF increased with age
With some pts with VWD type 1 normalizing their factor levels

103
Q

Micro-thrombocytopenia

A

Wiskott- Aldrich syndrome

104
Q

Macro-thrombocytopenia

A

VWD 2B
May Heggalin
Bernard-Soulier Syndrome
MDS
ITP

105
Q

Duration of post surgery AC prophylaxis in cancer pts

A

28 days

106
Q

EULAR vs Sapporo criteria for APLS

A

EULAR harder to use
More specific but less sensitive
More emphasis on IgG
Microvascular, valvular added
VTE/ATE in the context of predisposing conditions is not considered as criteria.

107
Q

Contraindication for argatroban in HIT

A

Bili > 1.5

108
Q

Contraindication for bivalirudin in HIT

A

CrCL<30

109
Q

Dose adjustment in edoxaban

A

CrCl < 50
Weight < 60 kg

110
Q

When to test for thrombophilia after VTE

A

When mild provoking factor- pregnancy, OCP, non surgical major risk
Chck only when considering Tx cessation
Don’t check during acute VTE

111
Q

When to test for thrombophilia in family members

A

PC, PS, AT

112
Q

When to test for thrombophilia before OCP

A

FHx + PC, PS, AT
Do not check if FHx w/o known thrombophilia

113
Q

When to test for thrombophilia in cancer pts

A

Low-int risk by Kohara score
+
FHx

Test panel and give prophylaxis if positive

114
Q

Thrombophilia testing in splanchnic and sinus vein thrombosis

A

Test for thrombophilia to guide Tx duration

115
Q

Drug associated TMA

A

Gemzar
CNI
Plavix

116
Q

Administration route of Tx for PNH

A

Ecu/Razviluzumab- IV
Pegcetacoplan- SC
Iptacopan (factor B)/Danicopan (factor D)- PO

117
Q

Prevelance of APLS in unprovoked PE

A

10%

118
Q

CAPS definition

A

3 organs
In 1 week
With APLA

119
Q

Severe FVII deficiency definition

A

< 10%
No good corelation between levels and bleeding

120
Q

Severe FXI deficiency definition

A

<15%
No good corelation between levels and bleeding

121
Q

CD55 and CD59 relation to complement

A

CD55- C3- extravascular
CD59- C5-9- intravascular

122
Q

Clone size effect on PNH outcomes

A

Larger clones are related to hemolysis and thrombosis

Erez's HEM TEST notes 2024 (35 decks)

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