Thrombosis facts Flashcards
1
Q
Rovelizumab vs Eculizumab in PNH
A
Same effectiveness
Revu given once every 8 weeks vs 2 weeks with eco
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
3
Q
Which C5 inhibitor is approved in pregnancy
A
Ecu
Does not cross placenta in high concentration
4
Q
Anticoagulant effect on LAC
A
DOACs cause false positive
5
Q
Definition of TTP excerbation
A
PLT < 150
30 days after cessation of PEX or caplacizumab
6
Q
Predictors of TTP exacerbation/relapse
A
low ADAMTS13
high Ab titers
black race
7
Q
Only treatment shown to reduce exacerbation in TTP
A
Caplacizumab
8
Q
Exacerbation rate after treatment with PEX in TTP
A
20-30%
9
Q
Prednisone tapering in TTP
A
Quickly over 4 weeks
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
11
Q
Caplacizumab duration of Tx
A
4 weeks
Can prolong to 8 weeks if ADAMTS13 still < 10%
12
Q
Interval of FU in TTP
A
1/w for a month
1/m for 3-6 months
1/ 3-6 months
13
Q
Threshold for preemptive Tx in TTP
A
ADAMTS13 < 10%
13
Q
Other non rituximab Tx in TTP
A
CSA- mildly effective, prolonged Tx needed
Splenectomy (not during acute exacerbation)- 70-80% ORR
14
Q
Neurocognitive late effects of TTP
A
Neurocognitive impairment- 60%
Mild depression- 80%
MDD- 20%
15
Q
DOACs in cancer associated VTE
A
Less thrombosis with more bleeding
16
Q
Thromboprophylaxis with DOACs in cancer
A
Apixaban- positive study 4% vs 10%
Rivaroxaban- negative study
17
Q
APLS M:F
A
1:5
18
Q
CAPS mortality
A
30% with AC, Steroids and PEX
19
Q
Recurrent thrombosis % in APLS pts on VKA
A
2-5%
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
21
Q
Management of microvascular complications in APLS
A
Aspirin
Immunosuppressive Tx
Complement inhibition
22
Q
ESRD in aHUS
A
66%
Probably better with current Tx
23
Q
PLASMIC score
A
PLT < 30
Hemolysis
No active cancer
No Hx of solid trasplant
MCV< 90
INR < 1.5
Cre < 2
24
Tx of pregnant aHUS pts
Eculizumab
need to increase dose
25
Managment of anti-C5 Tx in aHUS pts with infection/surgery
Continue Tx with anti-C5 and ABx
26
Tx of choice in acute aHUS
Eculizumab
Preferred over ravulizumab
Better when diagnosis not certain
Faster renal recovery
27
Median time to PLT recovery in aHUS
10 days
28
Monitoring response to Tx in aHUS
Total complement activity [CH50] < 20%) after 7 days- not possible with ravulizumab
as well as LDH, CBC normalization and Cre improvemet
29
Risk of relapse after anti-c5 discontiuation in aHUS
30-60%
Higher in CFH
30
Prophylactic ABx in aHUS
Penicillin during c5 inhibition
Vaccination is important but not enough
31
Paget Schroetter syndrome
Effort-induced thrombosis of the axillary and subclavian veins
compression of the subclavian vein at the thoracic outlet
32
APL ab presence
50% pts with SLE
5% of normal population
33
Difficulty with heparin Tx in APLS
aPTT may be elvated due to LAC
monitor through anti-Xa
34
Dosing of UFH
80 U/kg bolus
18 U/kg/h maintenence
Goal aPTT of x1.5-2
35
VWD type 2A
Absence of intermediate and high molecular weight multimers
36
VWD type 1C
increased clearence of VWF
Resembling type 1
but short response to DDAVP
37
VWD type 2B
Increased binding to PLT
thrombocytopenia
DDAVP is contraindicated bcs can worsen thrombocytopenia
38
VWD type 2M
Decreased affinty to PLT
39
VWD type 2N
Reduced binding to FVIII
FVIII very low
only type 2 that has high VWF Act/Ag (>0.7)
"NEIGHT"
"I 8 a sNake"
40
Limitations for VWF testing
VWF and FVIII are acute phase reactants
41
Significance of low dose ristocitin assay
Will show platelet aggregation in VWD type 2B
42
Diagnosis of VWD type 1
VWF Ag and Act <30
or <50 if bleeding symptoms
Increase after DDAVP
Act/ag >0.7
43
How to discriminate between VWD type 1 and type 1C
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
44
VWD 2B diagnosis
Low dose ristocitin causes agglutination in concentrations not usually able to cause activation
45
VWD 2B Tx
VWF
46
Platelet type VWD
Mutation in PLT causing enhanced binding to VWF
Tx with PLT
VWF is not effective because VWF will not be able to activate PLTs
47
VWD type 2N diagnosis
VWF:FVIII binding assays
May be falsely classified as mild hemophilia A
AR inhertiance or no response to FVIII Tx should raise the suspicion
48
Heide syndrome VWD type
2A
Loss of HMWM
49
Hemophilia severity
Mild < 40%
Moderate < 5%
Severe <1%
50
aPTT measurement method
Clot formation after incubaiton with phospholipid, calcium, and a contact activator
51
Effect of emicizumab on PTT
Shortens it substantially
52
FVIII concentrate dosing
1U/kg increases 2% activity
50U/kg is expected to raise levels to 100%
53
FIX concentrate dosing
1U/kg increases 1% activity
100U/kg is expected to raise levels to 100%
54
Laboratory goal of prophylaxis in hemophilia
Factor > 1%
55
Tx of choice for hemophilia pts with ab and breakthrough bleeding on emicizumab
rFVIIa
56
Clinical criteria of APLS
ATE
VTE
microvascular- livedo, nephropathy, PAH, adrenal hemorrhage, MI
Cardiac valve
Obstetric
Thrombocytopenia
57
New APLS criteria
EULAR 2023
3 points clinical
3 points labratory
58
When to stop anti-PLT before surgery
Prasugrel 7 days
Clopidogrel, Ticagrelor- 5 days
Usually no need for bridging
59
When to stop AC before surgery
High risk- 2 days
Low risk- 1 day
Except Dabigatran with CrCL<50 - 4 days
60
Kasabach-Merritt syndrome
Large hemangioma causing DIC
61
Which DOAC is a prodrug
Dabigatran
62
DOAC vs LMWH in cancer
Lower VTE with higher bleeding rate
Especially GI
Apixaban seems safest
63
VTE prophylaxis in ambulatory cancer pts
KHORANA score
When receiving chemo even if ambulatory- reduces mortality
commonly used only in high risk pts
64
Factors in Khorana score
Type of cancer
CBC
Obesity
65
DOAC as prophylaxis in cancer pts
Lower VTE with higher bleeding rate
Apixaban- AVERT
Rivaro- CASSINI
66
VTE in pregnancy incidence
1-2/100K
67
Coagulation factors that is decreased in pregnancy
PS
68
Which thrombophilia pts need prophylaxis during pregnancy
Homozygotes FVL/PT
Combined thrombophilias
PC/PS/AT with FHx
69
Which thrombophilia pts without FHx need prophylaxis postpartum
APLS- laboratory
AT
Homozygotes
70
Tx of pregnant women with laboratory APLS
Aspirin ante partum
LMWH postpartum
71
Tx of pregnant women with previous VTE
HIGHLOW study
low dose LMWH as efficient as intermediate dose
72
Risk of VTE in pregnancy for pts with previous estrogen provoked VTE
Ante partum >5%
post partum >10%
73
Prevalence of gestational thrombocytopenia
~5%
74
2nd line options in ITP during pregnancy
Azathioprine
Splenectomy
Anti-D?
75
Tx of TTP during pregnancy
PEX + Steroids
76
Contraindications for HRT
Smoker above 35 YO
Previous ATE
DM with complications
Uncontrolled HTN
Cirrhosis
Breast Ca
Migraine with neuro S/Sx
77
% of pts diagnosed with thrombophilia after pregnancy VTE
~50%
78
Conditions associated with
reduced VWF
Hypothyroidism
Blood type O
79
VWD types that are AR
2N
3
"N"ot to marry your sister (-;
80
Hemophilia A: Hemophilia B prevalence
Hemophilia A more prevelant
5:1
81
Titer of hemophilia Ab significance in Hemophilia A pts
>5 BU- high
<5 BU- low
in low titers factor replacement still possible
82
Glanzmann thrombasthenia platelet aggregation
Lack of response to agonists except ristocetin
83
Bernard-Soulier syndrome platelet aggregation
Lack of response to ristocetin with a response to all other agonists
84
Congenital bleeding diseases with normal coagulation tests
FXIII
Dysfibrinogenemia
Functional platelet defects
Collagen disorders
85
Worst mutation in aHUS
Factor H, Factor B- consider continuous C5 inhibition
86
CAPS incidence
1% of APLS
2:1 F:M
50% as first APLS diagnosis
~50% moratlity
87
Tx of CAPS
AC + Steroids +- IVIG/PEX (depending on severity)
88
Tx of ESUS
Aspirin
Rivaroxaban not more effective and more bleeding
89
Factor XI inhibitor in AF
Trial terminated early due to higher thromobosis compared to apixaban
90
Aspirin vs LMWH as prophylaxis after fractures
Same efficacy
Dose was bid (for both) which I don't understand
91
Efanesoctocog alfa
Novel Tx of severe hemophilia A
inhibits VWF clearance of FVIII
allowing FVIII a longer half life
reduces bleeding event rates
92
Factor XI inhibitor in post TKR surgery thromboprophylaxis
Same efficacy with less bleeding
93
C3 inhibition in PNH pts
90% ORR
for pts failing C5 inhibition
And also 1st line
94
Cocizumab
TFPI inhibitor
Effective in hemophilia A and B with inhibitors
vs no prophylaxis
reduced bleeding
95
Tranxemic acid in prehospital management of trauma pts
No functional benefit of pre + post
hospital administration
96
Tranexamic acid pre surgery
Reduces bleeding
97
Gray PLT syndrome
Low/absent of alpha granules
Progressive thrombocytopenia with large hypogranular PLT
PMF
98
Chediak Higashi syndrome
Infections
Albinism
Giant granules in neutrophils
Thrombocytopathy
Neuropathy
99
May-Hegglin anomaly
Macrothrombocytopenia
Dohle bodies in granulocytes
Hearing loss
Cataract
Renal failure
100
Wiskott-Aldrich syndrome
X linked
Immunodeficiency
Thrombocytopenia- low MPV
Eczema
101
FXI inhibtor after knee surgery
As effective as LMWH
102
Effect of aging on vWF
vWF increased with age
With some pts with VWD type 1 normalizing their factor levels
103
Micro-thrombocytopenia
Wiskott- Aldrich syndrome
104
Macro-thrombocytopenia
VWD 2B
May Heggalin
Bernard-Soulier Syndrome
MDS
ITP
105
Duration of post surgery AC prophylaxis in cancer pts
28 days
106
EULAR vs Sapporo criteria for APLS
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
Contraindication for argatroban in HIT
Bili > 1.5
108
Contraindication for bivalirudin in HIT
CrCL<30
109
Dose adjustment in edoxaban
CrCl < 50
Weight < 60 kg
110
When to test for thrombophilia after VTE
When mild provoking factor- pregnancy, OCP, non surgical major risk
Chck only when considering Tx cessation
Don't check during acute VTE
111
When to test for thrombophilia in family members
PC, PS, AT
112
When to test for thrombophilia before OCP
FHx + PC, PS, AT
Do not check if FHx w/o known thrombophilia
113
When to test for thrombophilia in cancer pts
Low-int risk by Kohara score
+
FHx
Test panel and give prophylaxis if positive
114
Thrombophilia testing in splanchnic and sinus vein thrombosis
Test for thrombophilia to guide Tx duration
115
Drug associated TMA
Gemzar
CNI
Plavix
116
Administration route of Tx for PNH
Ecu/Razviluzumab- IV
Pegcetacoplan- SC
Iptacopan (factor B)/Danicopan (factor D)- PO
117
Prevelance of APLS in unprovoked PE
10%
118
CAPS definition
3 organs
In 1 week
With APLA
119
Severe FVII deficiency definition
< 10%
No good corelation between levels and bleeding
120
Severe FXI deficiency definition
<15%
No good corelation between levels and bleeding
121
CD55 and CD59 relation to complement
CD55- C3- extravascular
CD59- C5-9- intravascular
122
Clone size effect on PNH outcomes
Larger clones are related to hemolysis and thrombosis
