Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Benign Heme Review > Deck 2 > Flashcards

Deck 2 Flashcards

1
Q

Dx for bleeding disorder with an elevated PT that completely corrects with PT mix

A

Factor VII deficiency—tx with rFVIIa prior to invasive procedures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Why is a negative family history common in ppl with factor VII deficiency?

A

It is autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Elevated PT/PTT with complete correction and normal thrombin time suggests what?

A

Common pathways deficiency but NOT fibrinogen. The remaining factors are II, V, and X

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the treatment for factor II deficiency?

A

Prothrombin deficiency; tx = PCC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How long to hold DOAC, assuming normal renal function, prior to minor surgery? Major?

A

2 days prior to minor; 3 days prior to major

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Assuming normal renal function, how many days after minor surgery until restarting DOAC? Major surgery?

A

1 day after for minor, 2 days after for major

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Assuming normal renal function, for how many days before and after minor surgery should a DOAC be held? Major?

A

2 days before minor, restart 1 day after; for major stop 3 days prior and restart 2 days after

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Dosing of apixaban and rivaroxaban for extended AC after 6 months of full dose AC

A

Apixaban 2.5 mg bid
Rivaroxaban 10 mg daily

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the main difference in MOA of LMWH and fondaparinux?

A

LMWH inhibits Xa AND IIa (thrombin) by potentiating antithrombin but fondaparinux only inhibits Xa also longer half life (4 vs 18 hr)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How does LMWH inhibit IIa?

A

By potentiating antithrombin (antithrombin III— nb IIa is thrombin) main effect is on Xa though

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

For patients on LMWH what is the ideal timing to check anti-Xa level? Therapeutic range?

A

3-4 hours after injection; 0.6-1.2 U/mL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the therapeutic range for twice daily LMWH vs once daily on anti-Xa? When should you check level?

A

0.6-1.2 for twice daily; 1-2 for once daily; either way should check 3-4 hours after injection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

In a patient who cannot be on other anticoagulants other than LMWH who develops recurrent thrombosis on LMWH what is the mgmt?

A

Increase dose 25-33%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How many points on 4T are considered low risk?

A

<3 points (<5% probability)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How many points on 4T is intermediate risk for HIT

A

4-5 points (~14% probability)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How many points on 4T is high risk of HIT?

A

6-8 (~64% probability)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

On the 4T score there are a max of 8 points and 3 general categories- low risk, intermediate risk, and high risk. What are the general probabilities associated with each?

A

Low risk <5%
Intermediate 14%
High risk 64%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the 2 available functional HIT assays?

A

Serotonin Release Assay (SRA) and heparin-induced platelet activation (HIPA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the 2 parenteral DTIs for HIT?

A

Argatroban safe in renal impairment
Bivalrudin safe in hepatic impairment (sounds like bilirubin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the clinical criteria for pregnancy comorbidity in APS?

A

One or more fetal deaths > 10th week gestation

One or more deaths before 34th week due to eclampsia, preeclampsia, or placental insufficiency

Three or more deaths <10th week

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

If there is concern for PT/INR confounding due lupus anticoagulant what else can be used to monitor warfarin efficacy? Therapeutic range?

A

Chromogenic Factor X; 20-40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

When might you use a chromogenic factor X assay for warfarin? Therapeutic range?

A

If concern about nonreliable PT/INR ie due to lupus inhibitor; 20-40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

MC MPN associated with Budd Chiari

A

Polycythemia Vera

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

In PNH there is an acquired mutation in which gene?

A

PIGA (phosphotidylinositol-glycan class A)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Which vitamin K dependent factor has the shortest half life?

A

FVII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What are the factors inhibited by warfarin and what pathways are they in?

A

II (common), VII (extrinsic PT), IX (intrinsic PTT), X (common) and then protein C/S

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Protein C and its cofactor, protein S, break down what factors

A

Factor V (common) and factor VIII (intrinsic PTT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Mgmt of INR > 10 not bleeding

A

Vitamin K PO, hold next dose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Mgmt of an SVT 5 cm In length or less than 3 cm away from saphenofemoral junction

A

45 days LMWH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Prothrombin Roman numeral

A

Factor II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What factors may cause an isolated elevation of PTT that corrects with mixing?

A

VIII, IX, and XI (Hem A, B, and C) and XII, prekallikrein and HMWK

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Suspicion in a pt with baseline moderate hemophilia A with baseline 6% who undergoes surgery and presents a few weeks later with joint bleed and factor <1%

A

Development of inhibitor in response to factor administration; new level is lower than baseline

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Another way FEIBA may show up on an exam as an option for bypassing agents is ______

A

Activated PCC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

For rapid acting factor repletion (not long acting Fc or pegylated) what is the dose of FIX repletion? FVIII?

A

FIX concentrate is 1u/kg to raise 1%
FVIII is 0.5 u/kg to raise 1%

For subsequent dosing is 1x or 0.5x (desired-actual) but for 100% repletion in severe hemophilia that is assumed to be 100-0

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Genetics of FXI def (Hemophilia C)

A

Autosomal Dominant—usually Ashkenazi Jews

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Mgmt of FMHx of VTE with pt who is homozygous prothrombin 20210 gene mutation in pregnancy

A

Antepartum and postpartum LMWH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Activated C, with its cofactor _____, cleaves which factors?

A

Protein S— cleave FV (common pathway) and FVIII (intrinsic; PT pathway)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Normally, factors V and VIII are cleaved by _______

A

Activated Protein C (with its cofactor Protein S)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What is a type I protein C deficiency?

A

Low protein C activity and low protein C antigen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is type II protein C deficiency?

A

Low protein C activity with normal antigen (qualitative defect)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Differentiate type I and II protein C deficiency

A

Type I low protein C activity and low antigen; Type II low activity and normal antigen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Reversal agent of dabigatran

A

Idarucizimab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

TAFI is converted to TAFIa by ______

A

Thrombin! It is thrombin activatable fibrinolysis inhibitor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What molecule acts by cleaving C-terminal lysine residues from fibrin fragments and in so doing disrupts recruitment of plasminogen

A

TAFI (Thrombin Activatable Fibrinolysis Inhibitor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

How does TAFI work?

A

Cleaves C terminal lysine residues from fibrin fragments and decreases recruitment of plasminogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Patients with elevated TAFI levels are prone to ______

A

Thrombosis as TAFI cleaves lysine residues from fibrin so that plasminogen cannot get in there

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Dabigatran prevents conversion of ____ to ______

A

Fibrinogen to fibrin as it is a direct thrombin (IIa) inhibitor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

In DIC these are the platelet, PT/PTT goals, and fibrinogen goals

A

Platelet > 50k, FFP for PTT/PT > 1.5x ULN, fibrinogen > 150

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Non eponymous name for trousseau syndrome

A

Chronic DIC (compensated DIC or low-grade DIC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What cancer is mostly commonly associated with chronic DIC (Trosseau syndrome)

A

Pancreatic adenocarcinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

How can VWF and FVIII help to differentiate liver disease from DIC

A

Will be low in DIC but preserved in liver failure bc made in endothelium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Inheritance of type I VWD? Type III?

A

Type I = AD; Type 2 = AR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Decreased high molecular weight multimers implies which type of VWD

A

2A or 2B —2B will have thrombocytopenia and ⬆️ RIPA but need genetic testing really

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Which type 2 VWD has preserved activity to antigen ratio?

A

2N bc there is normal VWF-Plt function but the FVIII binding site is issue so functionally they are like hemophilia A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

A VWF activity:antigen ratio < 0.7 with normal multimers suggests ____

A

Type IIM VWD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

PTT mixing study with partial correction and subsequent prolongation with incubation is absolutely characteristic of ______

A

Factor VIII inhibitor (Acquired Hemophilia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

What pattern will lupus anticoagulants give on a mixing study?

A

Inhibitor pattern

58
Q

What acquired factor inhibitor has been associated with fibrin glues and topical thrombin?

A

Factor V (common pathway)

59
Q

A pt with ESLD and fibrinogen of 50 needing paracentesis should be managed how?

A

Transfuse cryo to fibrinogen > 100

60
Q

Where is the Factor XIIIA subunit synthesized?

A

Megakaryocytes so in addition to FVIII and VWF, this too is normal in ESLD

61
Q

FMHx of thrombocytopenia and leukemia is associated with germline mutations in what 5 genes?

A

DDX41, ETV6, RUNX1, CEBPA, or ANKRD26

62
Q

What are the two TPO-RAs for periprocedural thrombocytopenia mgmt in ESLD

A

Avatrombopag and lusutrombopag

63
Q

FDA indicated use of lusutrombopag

A

Treatment of thrombocytopenia in pts with ESLD who are going to have a procedure

64
Q

FDA indication for avatrombopag

A

Treatment of thrombocytopenia in chronic liver disease patients requiring procedure

65
Q

3 FDA approved indications for eltrombopag

A

ITP with insufficient response to steroids, ivig, or splenectomy

SAA

HCV to raise platelet count before starting antivirals

66
Q

What TPO-RAs are approved for chronic liver disease needing procedure? HCV before antiviral therapy?

A

Lusutrombopag and Avatrombopag; Eltrombopag

67
Q

What drug is the MC cause of DITP

A

Vancomycin followed by TMP-SMX and piperacillin

68
Q

Potential rescue treatment for a bleeding ITP refractory to IVIG and steroids?

A

Anti Rh(D) immune globulin if Rh (+) and still have a spleen

69
Q

What second line ITP treatment will preclude the use of WinRho in the future?

A

Splenectomy as it leads to hemolysis in spleen

70
Q

First consideration for thrombocytosis in pt with hereditary hemorrhagic Telangiectasia? 3 genes

A

Iron deficiency with secondary thrombocytosis… ENG, ACVRL1, SMAD4

71
Q

2nd MC driver mutation in ET

A

CALR (JAK2 V617F > CALR > MPL)

72
Q

Though this agent can be used upfront in TTP what should be added to recurrent episodes in ppl who only got PLEX+steroids the first time

A

Rituximab 375 mg/m2 weekly x4

73
Q

Maximum dose romiplostim

A

10 ug/kg/week

74
Q

In theory, functional testing for HIT (SRA or HIPA) is not needed when the ELISA OD is >_____

A

2 as it is so likely at that point that a negative SRA is likely to be a false positive

75
Q

If HIT is diagnosed but no clinical evidence of clot what should you do, diagnostically?

A

4 limb US to look for clot

76
Q

This disease has mutations in GPIIB/IIIA

A

Glanzmann Thrombasthenia (fibrinogen receptor issue)

77
Q

What platelet disorder has severely diminished aggregation for all agonists except ristocetin?

A

Glanzmann Thrombasthenia GPIIB/IIIA defect (platelet fibrinogen receptor)

78
Q

Glanzmann Thrombasthenia testing will show lack of aggregation to all agonists except _____

A

Ristocetin

79
Q

What is the hemostasic defect in Hermansky Pudlak Syndrome?

A

Platelet Dense Granule Storage Pool deficiency (transfuse platelets and give DDAVP)…. Pulmonary fibrosis, oculocutaneous albinism, Hispanic

80
Q

Romiplostim and eltrombopag have response rates of approximately what in 2L ITP?

A

80%

81
Q

Is rituximab FDA approved for ITP?

A

No

82
Q

How do you manage brain bleed in person on aspirin plavix who is not undergoing surgery?

A

Antifibrinolytics (aminocaproic acid or tranexamic acid) and NOT platelet transfusions which may actually worsen outcomes

83
Q

What to suspect in a person who cannot achieve therapeutic PTT on heparin gtt? Mgmt?

A

Antithrombin deficiency; give FFP or antithrombin concentrate

84
Q

Mgmt of person with antithrombin deficiency who needs heparin gtt

A

If cannot get therapeutic PTT then either FFP or antithrombin concentrate

85
Q

Frequency of ppx FXIII concentrate infusion for FXIII def

A

Monthly Corifact

86
Q

DDx for a pt with bleeding disorder who has a normal PT, PTT, and PFA-100

A

Blood vessel abnormality including collagen vascular disease, FXIII def, or abnormal fibrinolysis

87
Q

Why is there an increased risk of brain abscess in Hereditary Hemorrhagic Telangiectasia? 3 Genes

A

Hematogenous Seeding from pulmonary AVM; ACVRL1, ENG, SMAD4

88
Q

Etiology of prolonged thrombin time that corrects with mixing study

A

Hypofibrinogenemia

89
Q

What type of hemophilia is autosomal dominant?

A

Hemophilia C (Factor XI) Ashkenazi Jews

90
Q

What coagulation factor is in alpha granules?

A

Factor V

91
Q

In addition to the endothelium, where else does VWF come from?

A

Platelet alpha granules

92
Q

Where does Platelet Factor 4 (PF4) come from?

A

Alpha Granules of platelets

93
Q

What is one of the functions of serotonin from dense granules in platelets?

A

Vasoconstrictor

94
Q

What are the two mechanisms of platelet adhesion in primary hemostasis?

A

GpIb-IX binding to VWF and GPVI binding collagen

95
Q

When are platelet GpIIb/IIIa receptors exposed on platelets?

A

When they are activated ie after adhesion they undergo shape change (ie after GPIb/IX binds VWF or GPVI binds collagen)

96
Q

Why does platelet activation play an important role in coagulation?

A

They provide an anionic aminophospholipid surface the coag cascade

97
Q

Classic microthrombocytopenia

A

Wiskott Aldrich syndrome (WAS)

98
Q

What may be the hemostatic defect in Chediak Higashi syndrome?

A

Dense granule deficiency (LYST) similar problem as Hermansky Pudlak

99
Q

The PFA-100 has blood exposed to a membrane containing what?

A

Collagen + Epinephrine and then if abnormal Collagen + ADP

100
Q

Explain the steps in the PFA-100

A

If collagen/epi time prolonged then go to collagen/ADP… if collagen/epi abnormal but collagen/ADP normal likely aspirin; if both prolonged then possible platelet defect or VWD

101
Q

What are the 5 agonists used in platelet aggregometry

A

Collagen, arachidonate, epinephrine, ADP, and ristocetin

102
Q

Explain the X and Y axis on platelet aggregation

A

Y axis is light transmission from 0 to 100 so the sample is turbid and as the platelets aggregate more light goes through

103
Q

How does arachidonate work as an agonist in platelet agg?

A

It gets converted to thromboxane A2 by cyclooxygenase (COX)

104
Q

Explain this platelet agg

A

Only aggregate to ristocetin = classic for Glanzmann—note there is more light transmission at the bottom of Y axis

105
Q

How does ristocetin work as an agonist in platelet aggregometry?

A

Causes GP1b-VWF dependent platelet agglutination; hence in Glanzmann where there is no GpIIB/IIIA receptor it is the only thing that works

106
Q

Explain this platelet agg

A

Bernard Soulier as there is NO response to ristocetin which causes VWF-GPIb aggregation but in BS there is no GPIb/IX

107
Q

Lack of a second wave to ADP and epinephrine on platelet agg suggests what defect?

A

Storage pool disorder (ie Hermansky Pudlak)

108
Q

What is the next step for this platelet agg?

A

Lack of second wave to ADP and epinephrine = storage pool defect ➡️ do electron microscopy

109
Q

A deficiency of GPVI on platelet would lead to lack aggregation to what agonist

A

Collagen

110
Q

Flow cytometry for platelet disorders would show lack of which marker in Wiskott Aldrich syndrome?

A

CD43

111
Q

What genes are mutated in Glanzmann?

A

ITGA2B and ITGB3

112
Q

What disease has mutated NBEAL2?

A

Gray platelet syndrome

113
Q

What disease has mutated ITGA2B or ITGB3?

A

Glanzmann (def GPIIB/IIIA)

114
Q

Reduced CD43 on platelet flow cytometry is seen in _____. Gene?

A

Wiskott-Aldrich (small platelets) WASP

115
Q

In addition to macrothrombocytopenia what would you see on a blood smear for MYH9 related diseases?

A

Dohle bodies in neutrophils

116
Q

What is THE major receptor for platelet aggregation?

A

GpIIB/IIIA

117
Q

What would be seen on a platelet agg for gray platelet syndrome? Gene?

A

Absent second wave bc storage pool def; NBEAL2

118
Q

For pregnancy associated ITP what are first line, second line, and late line options?

A

First line = steroids/IVIG

Second line = splenectomy (second trimester)

Late line = limited data (azathioprine, TPO-RA—none recommended but have been used)

119
Q

Mgmt of early pregnancy iron deficiency? Late?

A

Oral iron; IV Iron (iron sucrose)

120
Q

The heightened thrombotic risk post partum is traditionally defined as _____ weeks but may extend to a lesser extent to _____ weeks

A

6 weeks but may be some risk up to 13-15 weeks

121
Q

Mgmt of HIT in pregnancy

A

Fondaparinux or danaparoid

122
Q

Only exception to warfarin contraindication in pregnancy

A

Mechanical valves

123
Q

What heritable thrombophilia could be misdiagnosed during pregnancy? What heritable bleeding disorder could be missed?

A

Protein S deficiency as it drops in pregnancy; VWD bc VWF increases in pregnancy

124
Q

Definition of post partum hemorrhage

A

> 1000 mL blood loss or bleeding with SS of hypovolemia ; MC cause is uterine atony (70%)

125
Q

MC cause of post partum hemorrhage

A

Uterine Atony (70%)

126
Q

Young patients with monosomy 7 MDS are likely to have germline mutations in what gene?

A

GATA2

127
Q

Roman numeral for thrombin

A

IIa; II is prothrombin

128
Q

Alpha thalassemias are seen mostly in what geographic regions?

A

Western Africa and Southeast Asia

129
Q

What hemoglobin is hemoglobin barts?

A

Gamma4 seen in alpha thalassemia

130
Q

What is HBH?

A

Beta 4 tetramers, B4 will form Heinz bodies

131
Q

What is the abnormality in SCD?

A

Sixth amino acid in Beta globin is changed from glutamate (charged) to valine (hydrophobic)

132
Q

A beta globin with 6th AA glutamate to valine has

A

HbS

133
Q

A patient with beta globin that has 6th AA glutamate to lysine has

A

HbC

134
Q

What is the genetic abnormality in Hemoglobin C?

A

Beta globin 6th AA is glutamate to lysine

135
Q

What are the 3 main genotypes for Sickle Cell Dz?

A

SS, SC, and Sickle-B thalassemia

136
Q

MCHC in HbC dz?

A

Increased due to dehydration

137
Q

Hemoglobin E and HbA2 migrate together on what type of electrophoresis?

A

Alkaline AND Citric (Acidic)

138
Q

Given choices of HbE, HbA2, and HbC what happens on alkaline gel vs citric agar?

A

HbE and A2 comigrate on alkaline and acidic agar but C migrates differently on citric agar

139
Q

Is HbE more like a sickle disorder or a thalassemia?

A

Like B thalassemia as it decreases B globin synthesis

140
Q

Hemoglobin E is MC where?

A

India

141
Q

Though HbE and HbA2 comigrate on both citric and alkaline agar, how to differentiate?

A

You would never have a HbA2 as high as would be seen on electrophoresis for HbE dz

142
Q

Hemoglobin Lepore is in what geographic region?

A

Balkans

Benign Heme Review (2 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions