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Hematology-Oncology > Assessment 2 > Flashcards

Assessment 2 Flashcards

1
Q

What are indications for transfusion in patient with sickle cell?

A

-pregnancy NOT in itself a cause

  • CV compromise
  • frequent pain episodes
  • CNS events
  • Inability to maintain adequate cell production as measured by retic count
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2
Q

What are the clinical/imaging signs of acute chest syndrome?

Causes?

How should you treat it?

A

imaging - new pulmonary infiltrate on CXR

clinical

  • chest pain
  • fever
  • tachypnea
  • wheezing or cough

Causes

  • Pneumonia: Strep pneumo, mycoplasma, chlamydia, viruses
  • bone infarction w/ fat embolism

Tx

  • supplemental O2
  • antibiotics
  • erythrocyte exchange transfusion
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3
Q

Diamond-Blackfan anemia

  • inheritance
  • defect
A
  • autosomal dominant
  • ribosomal defect -> increased apoptosis leading erythroid failure
  • pure red cell aplasia
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4
Q

Fanconi anemia

  • inheritance
  • defect
  • inc risk of
A
  • almost always autosomal recessive
  • DNA repair defect -> bone marrow failure
  • inc risk of cancer
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5
Q

What are 2 coagulation proteins synthesized by megakaryocytes?

which 2 are endocytosed into the meg?

A

Synthesized

  • factor V
  • vWF

Endocytosed

  • fibrinogen
  • plasminogen
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6
Q

What are 3 inhibitors of the hemostatic system (prevent clots)

A

– Antithrombin and endogenous heparins
– Tissue factor pathway inhibitor (TFPI)
– Proteins C and S

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7
Q

Compare the 2 platelet glycoproteins

GP VI vs. GP Ib/IX/V

A

GP VI

  • Adhesion under LOW FLOW CONDITIONS
  • collagen receptor

GP Ib/IX/V

  • Adhesion under HIGH FLOW CONDITIONS
  • vWF receptor
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8
Q

von Willebrand factor

  • stored in
  • cleaved by
  • carries
A

stored in weibel-palade bodies in endothelial cells AND alpha granules of platelets

cleaved by ADAMTS13

carries factor VIII (antihemophilic) and protects it from degradation

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9
Q

Anti-thrombotic factors produced by normal endothelial cells

A

– Prostacyclin (PGI2)
– ADPases
– Heparins
– Thrombomodulin

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10
Q

Mucocutaneous bleeding is associated with which hemostatic defect?

A

PRIMARY

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11
Q

Name the following coag factors

I, II, III, IV, VIII, XII, XIII

A
  • I Fibrinogen
  • II Prothrombin
  • III Thromboplastin
  • IV Calcium
  • VIII Antihemophilic
  • XII Hageman
  • XIII Fibrin stabilizing
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12
Q

Vitamin K dependent factors (use mnemonic)

Why is it needed?

which drug blocks the production of these factors? how?

What else in vitamin K dependent?

A

1972

X, IX, VII, II

Vit K is cofactor for glutamyl carboxylase

  • warfarin -> inhibits the gamma carboxylation in the synthesis of these factors
  • prevents Ca2+ dependent binding of phospholipids

Anti-coagulation proteins C and S
-this is why need to give heparin bridge when starting tx with warfarin

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13
Q

Heparin MoA

A

potentiates antithrombin III -> inactivates IIa (thrombin), VIIa, IXa, Xa, XIa, XIIa

Also kallikrein

7+2 = 9,10,11,12

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14
Q

What vasodilates and increases vascular permeability in secondary hemostasis?

A

Bradykinin -> cleaved from high MW kinin

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15
Q

Which factors are deficient in hemophilia A and B

A

Hemophilia A - factor VIIIa

Hemophilia B = factor IXa (christmas disease)

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16
Q

What does the prothrombinase complex consist of? What does it form?

A

Xa + Va -> converts II to IIa (thrombin formation)

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17
Q

Which steps in coag cascade require Ca2+

hint: there are 5

A

Formation of

  • VIIa
  • IXa
  • Xa
  • IIa

-note that these are also vit K dependent factors

Also for formation of cross-linked fibrinogen

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18
Q

Which factor is bound to the vWF in circulation? vW disease will lead to an increase in PT or PTT?

A
  • Bound to factor VIII
  • increase in PTT (intrinsic pathway)
  • factor VIIIa is a cofactor that works with factor IXa to form factor Xa

most severe deficiency seen with type 3

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19
Q

Which platelet receptor (integrin) binds to fibrinogen?

A

GP IIb/IIIa

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20
Q

Which platelet integrin binds to collagen?

A

GP Ia/IIa

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21
Q

What activates factor XIII?

A

Thrombin (IIa)

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22
Q

Coagulation is inhibited at what 3 points?

A

– Activation of fX by the TF/VIIa complex through TFP

– Activity of thrombin -> when bound to thrombomodulin increases protein C and TAFI activity and decreased activity of factors involved with coagulation

– Cofactor activity of VIIIa and Va -> inactivated by protein C and S (co-factor)

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23
Q

What activates protein C?

A

Thrombin-thrombomodulin complex

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24
Q

Antithrombin MoA

inhibits?

activity increased by?

A
  • serine protease inhibitor
  • inhibits activity of both Xa and thrombin (IIa)
  • activity increased by heparin (see other cards)
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25
Q

What degrades cross-linked fibrin (fibrinolysis)? What activates this enzyme?

A

plasmin

-plasminogen converted to plasmin by tPa and uPA

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26
Q

3 things that INHIBIT fibrinolysis

A
  1. alpha2-antiplasmin -> direct inhibition of plasmin
  2. Plasminogen activator inhibitor 1 (PAI-1)
  3. TAFI (also see other card)
    - cleaves C-terminal lysine residues from fibrin
    - This prevents plasmin and tPA from binding
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27
Q

INR measured which Vit K dependent factors?

normal range for people on warfarin?

A

II, VII and X

normal = 2-3

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28
Q

Diagnostic pentad for TTP

Deficiency in?

A
  • thrombocytopenia
  • microangiopathic hemolysis with schistocytes
  • Neurological sxs
  • Fever
  • Renal impairment

ADAMTS-13 deficiency - acquired or genetic

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29
Q

atypical HUS

  • trigger
  • most common manifestation
  • pathogenesis
A

trigger - URI or gastroenteritis in most patients
-sudden onset

MC manifestation -> renal insufficiency

path -> chronic uncontrolled activation of alternate pathway of complement
-most commonly defect in CFH -> highest death rate

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30
Q

FDA approved tx for aHUS?

A

Eculizumab - mAb against C5

  • blocks formation of MAC
  • blocks C5a formation
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31
Q

Mixing ime
-immediate acting vs delayed

Purpose of mixing studies

A

Immediate - lupus anticoagulant

delayed - factor inhibitors

Distinguish factor deficiencies from factor inhibitors

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32
Q

Normal values for the following

  • PT
  • PTT
  • TT
A
  • PT -> 10-12 seconds
  • PTT -> 30 - 45 seconds
  • TT -> 14 to 16 seconds
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33
Q

Russell viper venom measures which factors?

A

Factor X and down

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34
Q

Thromboxane A2 effects?

counterbalanced by?

A

Thromboxane A2 - potent mediator of platelet aggregation and vasoconstriction
-made in platelets

Counterbalanced by PGI2 made in endothelial cells

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35
Q

MC sx of antithrombin deficiency

A

venous thrombosis of lower extremity at early age peaking at 2nd decade of life

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36
Q

Acute blood volume loss at which transfusion is indicated

A

> 25-40%

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37
Q

Most immunogenic antigen

A

D

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38
Q

Clinical indications for DAT

A
  • autoimmune hemolytic anemia
  • transfusion anemia
  • hemolytic disease of fetus/newborn
  • drug induced Abs
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39
Q

restrict transfusion to Hb<7 except in which case?

A

MI or unstable angina

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40
Q

Opsonization vs directly lysis

  • extravascular or intravascular hemolysis
  • which one more severe
A

Opsonization - extravascular

Direct lysis - intravascular
-MORE SEVERE

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41
Q

What does cryoprecipitate contain?

indications?

A
  • Factor VIIIc
  • Factor VIII-vWF
  • factor XIII
  • Factor I (fibrinogen)

Indications
-fibrinogen deficiencies - DIC, dilutional coagulopathy

-factor VIII deficiency

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42
Q

Which coag factors does FFP contain?

Indications?

A

ALL OF THEM

  • liver failure, dilutional coagulopathy, DIC
  • reverse warfarin
  • replacement solution -> plasma exchange (a type of therapeutic apheresis procedure) for TTP
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43
Q

Indications for washed components

A

-remove most of plasma

Indications

  • prevent febrile non-hemolytic tx rxns
  • prevent urticarial allergic rxns
  • prevent anti-IgA anaphylaxis
44
Q

Etiology for febrile nonhemolytic rxn

A
  • recipient Ab to donor WBC
  • cytokines accumulated during storage component

sxatic tx

45
Q

Severe event in allergic rxns for transfusion due to

A

IgA deficiency - anti-IgA present

46
Q

Most common cause of transfusion associated deaths?

A

TRALI - transfusion related acute lung injury

47
Q

TRALI

  • time of onset
  • presentation
  • CXR shows
  • improves w/in
  • therapy (include ineffective tx)
  • etiology
A

-1 to 6 hours after transfusion

  • Acute respiratory distress
  • HypoTN unresponsive to fluid administration
  • CXR -> bilateral pulmonary edema
  • improves w/in 2 to 5 days

Supportive tx includes:

  • O2
  • intubation
  • mechanical vent

Ineffective tx

  • IV fluids
  • diuretics (NO NO)
  • steroids
  • IVIG -> damage already done

Etiology

  • donor HLA or PMN Abs
  • Abs can be recipient derived
  • rxn b/w endothelial cell and WBC -> Complement activation leading to endothelial damage and plasma release into lung parenchyma
48
Q

How to manage TACO patients (transfusion associated circulatory overload)

A

-diuretics

49
Q

TA-GvHD mortality due to?

Presentation of TA-GvHD

A

infection or hemorrhage 2ndary to pancytopenia

Presentation

  • fever
  • extensive erythematous rash
  • jaundice and liver dysfx
  • profuse water diarrhea
  • PANCYTOPENIA
50
Q

Gamma irradiation not indicated in? (2 things)

A

full-term neonates

HIV infected patients

51
Q

Post transfusion infection most common due to?

A

CMV

52
Q

Leukocyte reduction filters

  • target leukocyte count
  • remove
  • reduce risk of
A

target -> < 5 million

Remove

  • APCs
  • virus/bacteria laden leukocytes

Reduce risk of

  • febrile non-hemolytic transfusion reactions
  • platelet alloimmunization
  • infection
  • cytokine production
53
Q

Most common genetic defect predisposing to thrombosis

A

Factor V Leiden mutation
-Hz prevalence = 3-5%

12-20% of patients of unselected venous thrombosis

54
Q

Risk increase of VTE with prothrombin 20210A mutation

A

2-3 fold increase

55
Q

Who is at increased risk for hereditary thrombophilias? (list 4)

A
  • multiple family members w/ blood clots

- young age of onset ( CONTROVERSIAL and testing NOT RECOMMENDED

56
Q

Testing for FVL/PT in sxatic patients?

Sxatic family members of FVL/PT patients?

A

NOT RECOMMENDED

  • does not change outcome
  • harms can outweigh benefits
57
Q

What’s the most important factor when deciding about oral contraceptives

A

family hx of VTE -> find an alternative (e.g. patch)

58
Q

Incidence of hemophilia A vs B

Factor deficiency

Inheritance

Carriers symptomatic?

A

Hemophilia A -> 1/5k
-Factor VIII

Hemophilia B -> 20k
-Factor IX

X-linked recessive for BOTH

female carriers - 10% risk of sxs of hemophilia

59
Q

Severe phenotype of hemophilia due to which genotype?

What about moderate/mild?

A

Point mutation leading to STOP codons

also splice site mutations

Moderate/mild -> missense

60
Q

Most costly complication of hemophilia? How is it managed?

A

Allo Inhibitors - IgG Abs againsts fVIII and IX

Tx by

  • increasing dose
  • bypass the factors
  • DDAVP
61
Q

DDAVP

  • MoA
  • indications
A

MoA -> induces release of fVIII and vWF from patient’s endothelial cells and increases factor levels

Indications
-Mild to moderate hemophilia A

62
Q

Most common inherited bleeding disorder?

A

von WIllebrand disease

1-3%
M=F

63
Q

Most severe form of vWD

-inheritance?

A

type 3

  • no vWF
  • rare

AUTOSOMAL RECESSIVE
-also type 2N - vWF can’t bind to factor VIII (often misdxed w/ Hemophilia A)

64
Q

Virchow’s triad

A
  • flow/stasis
  • endothelial damage
  • hypercoagulable state

THROMBOSIS

65
Q

Anticoagulant drug of choice during pregnancy and why?

A

Heparin unfractionated - does not cross placenta

66
Q

Tx of severe UFH toxicity?

A

Protamine sulfate - heavily positively charged, binds UFH and prevents UFH-antithrombin III interaction

67
Q

UFH vs LMWH

LMWH is DOC for:

A

LMHW can ONLY inhibit Xa
-lacks binding site for IIa (thrombin)

ALSO
-LMWH -> less binding to plasma and EC proteins:

  • higher bioavailability
  • more predictable response
  • less patient to patient variations
  • less need for monitoring

LMWH is DOC for:

  • prophylaxis and tx of DVT/PE
  • management of acute coronary syndrome
68
Q

MoA of warfarin (coumadin)

A

Blocks vitamin K reductase -> prevents gamma carboxylation of vit K dependent factors
-X, IX, VII, II

Also proteins C and S

69
Q

How long does it take for reduction in circulating factors after warfarin tx initiated?

A

30-50% reduction 3 - 6 days AFTER start of tx

70
Q

Management of warfarin toxicity from mild to severe

A

-D/C warfarin
-D/C warfarin + vitamin K1
-D/C warfarin + fresh plasma with vitamin K1
supplementation

71
Q

List 3 new anticoagulants that block thrombin (IIa)

A
  1. Dabigatran (oral)
  2. Bivalirudin (parenteral)
  3. Argatroban (parenteral)
72
Q

synthetic pentasaccharide that works with AT to selectively inhibit Xa

A

Fondaparinux (parenteral)

73
Q

new factor X inhibitor

A

Rivaroxaban (oral)

74
Q

List 3 antiplatelet agents and MoA

A
  1. ASA -> irreversible inhibition of COX (decrease TXA2 production)
  2. Clopidogrel -> irreversible inhibition of ADP receptor (P2Y12)
    - rapid absorption
  3. Abciximab -> mAb againsts GpIIb/IIIa
    - used when placing stents

Final common mediator = GpIIb/IIIa

75
Q

Streptokinase

  • source
  • MoA
  • administration
A

Source - hemolytic streptococci
-risk for anaphylaxis

MoA -> exposes active site of plasminogen so more plasmin can be made

Administration -> IV bolus followed by infusion

76
Q

t-PA vs tenecteplase

A

Tenecteplase

  • increase half life -> single bolus dose NOT needed
  • increase activity
77
Q

Thrombolytic bleeding mechanism

A

Lysis of physiological thrombi

Plasmin mediated degradations of fV and VIII -> systemic lysis

78
Q

How to stop thrombolytic bleeding

A
  • D/C thrombolytic drug – short t1/2
  • Transfusion of plasma, whole blood, fibrinogen

Aminocaproic acid:
-binds to plasminogen and plasmin, blocks access to fibrin

79
Q

Artery vs arteriole fx based on wt/id ratio

A

Arteries -> very elastic

  • pressure containment
  • pressure storage

Arterioles
-resistance to flow!!!

80
Q

Venous return has direction relationship with?

A

Cardiac output

-regulate return of blood to the heart

81
Q

Arteriolar constriction has what effect on pressure in the capillaries?

Dilation?

A

DECREASES

INCREASES

82
Q

The principle that explains why aorta has to be so thick and capillaries don’t blow up is?

A

LePlace’s Law

Tension = Transmural pressure x radius

Aorta -> very elastic
capillaries -> very small

83
Q

Bernard-Soulier

  • inheritance
  • defect
  • platelet appearance of smear
  • labs
  • tx
A
  • autosomal recessive
  • defect in Gp1b
  • giant platelets w/ thrombocytopenia

Labs

  • inc BT
  • ristocetin aggregation absent

tx w/ platelet transfusion

ADHESION DEFECT

84
Q

Glanzmann’s thrombasthenia

  • inheritance
  • defect
  • platelet appearance of smear
  • labs
  • tx
A
  • autosomal recessive
  • defect in GpIIb/IIIa -> fibrinogen receptor
  • platelets look normal

LABS

  • increased bleeding time
  • Only ristocetin aggregation is NORMAL

Tx

  • platelet transfusion
  • hormone tx for menorrhagia
  • iron replacement tx

DEFECT IN PLATELET AGGREGATION
-much worse than bernard-soulier

85
Q

DEFECT IN PLATELET SECRETION

  1. Gray platelet syndrome
  2. Storage pool disease
  3. Hermansky-Pudlak Syndrome
A
  1. Gray platelet syndrome
    - alpha granules missing
    - large gray platelets
  2. Storage pool disease
    - delta granules missing
    - no ADP or serotonin
    - normal platelet morphology
    - tx w/ DDAVP and transfusion
  3. Hermansky-Pudlak Syndrome
    - variant storage pool disease
    - AR
    - oculocutaneous albinism
86
Q

May Hegglin anomaly

  • inheritance
  • characteristics
  • mutation
A

-AR

  • macrothrombocytopenia
  • DOHLE bodies in PMNs

-mutation in MYH-9

87
Q

Wiskott-Aldrich

A

microthrombocytopenia

X-linked recessive

Tx

  • irradiated platelets
  • bone marrow transplantation
88
Q

Heparin induced thrombocytopenia

  • path
  • complications
  • tx
A

PATH
-Abs againsts hep-PF4 complex -> activation of platelets

COMPLICATIONS
-venous+arterial thrombosis (venous more common)

TX

  • stop hep
  • refludin or argatroban -> thrombin inhibitor
89
Q

Which factor is always elevated w/ liver disease?

A

VIII:C

90
Q

Lupus like anticoagulant only causes bleeding when?

A

When there is concomitant thrombocytopenia or prothrombin deficiency

91
Q

Antiphospholipid Ab syndrome

  • criteria
  • labs
  • tx
A

-presence of cardiolipin Ab and/or lupus-like anticoagulant + any of the following:

A. thrombosis

B. recurrent miscarriages

TREATMENT
-life long anticoagulation

-for pregnancy
A. Prednisone + 1 baby ASA
B. Heparin + 1 baby ASA

DRAMATIC DROP IN MISCARRIAGES

92
Q

What is the inheritance for all inherited thrombotic disorders

  • which one has the highest increase in risk?
  • which one is most common?
A

AUTOSOMAL DOMINANT

ATIII deficiency -> highest risk (30x)

Factor V leiden -> most common

93
Q

Factor VIII:C inheritance vs. Factor VIII:Ag/vWF

A

Factor VIII:C inheritance -> sex-linked

Factor VIII:Ag/vWF -> autosomal

94
Q

MC inherited disorder of hemostasis?

A

von willebrand’s disease followed by hemophilia A

95
Q

MC bleeding manifestation of hemophilia?

A

Hemarthrosis

96
Q

What does cryoprecipitate contain?

A

fibrinogen
VIII
vWF
XIII

97
Q

Which arch develops in common and internal carotid arteries?

A

arch 3 + part of dorsal aorta

98
Q

right subclavian artery formed from?

brachiocephalic?

What happens to the right 6th arch?

A

4th arch + 7th intersegmental + intervening portion of dorsal aorta (RIGHT SIDE)

right aortic sac -> brachiocephalic

R 6th arch dissapears

99
Q

Left side vessel embryo

  • aortic arch
  • left subclavian
  • ductus arteriosus/ligamentum arteriosum
  • pulmonary arteries
A
  • aortic arch -> 4th arch + part of aortic sac
  • left subclavian -> L 7th intersegmental
  • ductus arteriosus -> left 6th arch: shunts blood from pulmonary trunk to aortic arch
  • pulm art -> left 6th arch near aortic sac
100
Q

Arterial system

  • ventral
  • lateral
  • posterolateral
A

ventral -> digestive tract + glands

lateral -> adrenal glands, gonads, kidneys

posterolateral -> body wall

101
Q

all 3 embryonic venous systems drain into the?

A

right/left horns of sinus venosus

102
Q

Double SVC is due to?

A

Persistence of left anterior cardinal vein

  • will drain into R atrium by way of coronary sinus
  • problem is gets into the left atrium
103
Q

2 causes of vascular rings

A
  1. double aortic arch
  2. abnormal right subclavian artery
    - due to abnormal obliteration of right 4th aortic arch
    - 7th intersegmental (r subclavian) retains connection with dorsal aorta
    - not usually a prob

COMPRESSION OF TRACHEA AND ESOPHAGUS

104
Q

What’s worse - preductal or postductal coarctation of the aorta?

A

Pre

105
Q

What needs to be present for atrial and ventricular partitions to form?

A

Septum intermedium - formed by sup and inferior walls of endocardial cushion coming together

106
Q

Aortic and semilunar valve formation

A

aortic valve -> right, left and posterior cusps

Pulmonary -> right, left and anterior cusps

107
Q

Pacemaker location in early development?

S-A node

A-v node

A
  1. ventricle
  2. right common cardinal or right sinus venosus
  3. from superior endocardial cushion

Hematology-Oncology (3 decks)

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