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Hematology-Oncology > Assessment 2 > Flashcards

Assessment 2 Flashcards

(107 cards)

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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
What degrades cross-linked fibrin (fibrinolysis)? What activates this enzyme?
plasmin | -plasminogen converted to plasmin by tPa and uPA
26
3 things that INHIBIT fibrinolysis
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
27
INR measured which Vit K dependent factors? normal range for people on warfarin?
II, VII and X normal = 2-3
28
Diagnostic pentad for TTP Deficiency in?
- thrombocytopenia - microangiopathic hemolysis with schistocytes - Neurological sxs - Fever - Renal impairment ADAMTS-13 deficiency - acquired or genetic
29
atypical HUS - trigger - most common manifestation - pathogenesis
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
30
FDA approved tx for aHUS?
Eculizumab - mAb against C5 - blocks formation of MAC - blocks C5a formation
31
Mixing ime -immediate acting vs delayed Purpose of mixing studies
Immediate - lupus anticoagulant delayed - factor inhibitors Distinguish factor deficiencies from factor inhibitors
32
Normal values for the following - PT - PTT - TT
- PT -> 10-12 seconds - PTT -> 30 - 45 seconds - TT -> 14 to 16 seconds
33
Russell viper venom measures which factors?
Factor X and down
34
Thromboxane A2 effects? | counterbalanced by?
Thromboxane A2 - potent mediator of platelet aggregation and vasoconstriction -made in platelets Counterbalanced by PGI2 made in endothelial cells
35
MC sx of antithrombin deficiency
venous thrombosis of lower extremity at early age peaking at 2nd decade of life
36
Acute blood volume loss at which transfusion is indicated
> 25-40%
37
Most immunogenic antigen
D
38
Clinical indications for DAT
- autoimmune hemolytic anemia - transfusion anemia - hemolytic disease of fetus/newborn - drug induced Abs
39
restrict transfusion to Hb<7 except in which case?
MI or unstable angina
40
Opsonization vs directly lysis - extravascular or intravascular hemolysis - which one more severe
Opsonization - extravascular Direct lysis - intravascular -MORE SEVERE
41
What does cryoprecipitate contain? | indications?
- Factor VIIIc - Factor VIII-vWF - factor XIII - Factor I (fibrinogen) Indications -fibrinogen deficiencies - DIC, dilutional coagulopathy -factor VIII deficiency
42
Which coag factors does FFP contain? | Indications?
ALL OF THEM - liver failure, dilutional coagulopathy, DIC - reverse warfarin - replacement solution -> plasma exchange (a type of therapeutic apheresis procedure) for TTP
43
Indications for washed components
-remove most of plasma Indications - prevent febrile non-hemolytic tx rxns - prevent urticarial allergic rxns - prevent anti-IgA anaphylaxis
44
Etiology for febrile nonhemolytic rxn
- recipient Ab to donor WBC - cytokines accumulated during storage component sxatic tx
45
Severe event in allergic rxns for transfusion due to
IgA deficiency - anti-IgA present
46
Most common cause of transfusion associated deaths?
TRALI - transfusion related acute lung injury
47
TRALI - time of onset - presentation - CXR shows - improves w/in - therapy (include ineffective tx) - etiology
-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
How to manage TACO patients (transfusion associated circulatory overload)
-diuretics
49
TA-GvHD mortality due to? Presentation of TA-GvHD
infection or hemorrhage 2ndary to pancytopenia Presentation - fever - extensive erythematous rash - jaundice and liver dysfx - profuse water diarrhea - PANCYTOPENIA
50
Gamma irradiation not indicated in? (2 things)
full-term neonates HIV infected patients
51
Post transfusion infection most common due to?
CMV
52
Leukocyte reduction filters - target leukocyte count - remove - reduce risk of
target -> < 5 million Remove - APCs - virus/bacteria laden leukocytes Reduce risk of - febrile non-hemolytic transfusion reactions - platelet alloimmunization - infection - cytokine production
53
Most common genetic defect predisposing to thrombosis
Factor V Leiden mutation -Hz prevalence = 3-5% 12-20% of patients of unselected venous thrombosis
54
Risk increase of VTE with prothrombin 20210A mutation
2-3 fold increase
55
Who is at increased risk for hereditary thrombophilias? (list 4)
- multiple family members w/ blood clots | - young age of onset ( CONTROVERSIAL and testing NOT RECOMMENDED
56
Testing for FVL/PT in sxatic patients? | Sxatic family members of FVL/PT patients?
NOT RECOMMENDED - does not change outcome - harms can outweigh benefits
57
What's the most important factor when deciding about oral contraceptives
family hx of VTE -> find an alternative (e.g. patch)
58
Incidence of hemophilia A vs B Factor deficiency Inheritance Carriers symptomatic?
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
Severe phenotype of hemophilia due to which genotype? What about moderate/mild?
Point mutation leading to STOP codons also splice site mutations Moderate/mild -> missense
60
Most costly complication of hemophilia? How is it managed?
Allo Inhibitors - IgG Abs againsts fVIII and IX Tx by - increasing dose - bypass the factors - DDAVP
61
DDAVP - MoA - indications
MoA -> induces release of fVIII and vWF from patient's endothelial cells and increases factor levels Indications -Mild to moderate hemophilia A
62
Most common inherited bleeding disorder?
von WIllebrand disease 1-3% M=F
63
Most severe form of vWD | -inheritance?
type 3 - no vWF - rare AUTOSOMAL RECESSIVE -also type 2N - vWF can't bind to factor VIII (often misdxed w/ Hemophilia A)
64
Virchow's triad
- flow/stasis - endothelial damage - hypercoagulable state THROMBOSIS
65
Anticoagulant drug of choice during pregnancy and why?
Heparin unfractionated - does not cross placenta
66
Tx of severe UFH toxicity?
Protamine sulfate - heavily positively charged, binds UFH and prevents UFH-antithrombin III interaction
67
UFH vs LMWH LMWH is DOC for:
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
MoA of warfarin (coumadin)
Blocks vitamin K reductase -> prevents gamma carboxylation of vit K dependent factors -X, IX, VII, II Also proteins C and S
69
How long does it take for reduction in circulating factors after warfarin tx initiated?
30-50% reduction 3 - 6 days AFTER start of tx
70
Management of warfarin toxicity from mild to severe
-D/C warfarin -D/C warfarin + vitamin K1 -D/C warfarin + fresh plasma with vitamin K1 supplementation
71
List 3 new anticoagulants that block thrombin (IIa)
1. Dabigatran (oral) 2. Bivalirudin (parenteral) 3. Argatroban (parenteral)
72
synthetic pentasaccharide that works with AT to selectively inhibit Xa
Fondaparinux (parenteral)
73
new factor X inhibitor
Rivaroxaban (oral)
74
List 3 antiplatelet agents and MoA
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
Streptokinase - source - MoA - administration
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
t-PA vs tenecteplase
Tenecteplase - increase half life -> single bolus dose NOT needed - increase activity
77
Thrombolytic bleeding mechanism
Lysis of physiological thrombi Plasmin mediated degradations of fV and VIII -> systemic lysis
78
How to stop thrombolytic bleeding
- 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
Artery vs arteriole fx based on wt/id ratio
Arteries -> very elastic - pressure containment - pressure storage Arterioles -resistance to flow!!!
80
Venous return has direction relationship with?
Cardiac output | -regulate return of blood to the heart
81
Arteriolar constriction has what effect on pressure in the capillaries? Dilation?
DECREASES INCREASES
82
The principle that explains why aorta has to be so thick and capillaries don't blow up is?
LePlace's Law Tension = Transmural pressure x radius Aorta -> very elastic capillaries -> very small
83
Bernard-Soulier - inheritance - defect - platelet appearance of smear - labs - tx
- autosomal recessive - defect in Gp1b - giant platelets w/ thrombocytopenia Labs - inc BT - ristocetin aggregation absent tx w/ platelet transfusion ADHESION DEFECT
84
Glanzmann's thrombasthenia - inheritance - defect - platelet appearance of smear - labs - tx
- 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
DEFECT IN PLATELET SECRETION 1. Gray platelet syndrome 2. Storage pool disease 3. Hermansky-Pudlak Syndrome
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
May Hegglin anomaly - inheritance - characteristics - mutation
-AR - macrothrombocytopenia - DOHLE bodies in PMNs -mutation in MYH-9
87
Wiskott-Aldrich
microthrombocytopenia X-linked recessive Tx - irradiated platelets - bone marrow transplantation
88
Heparin induced thrombocytopenia - path - complications - tx
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
Which factor is always elevated w/ liver disease?
VIII:C
90
Lupus like anticoagulant only causes bleeding when?
When there is concomitant thrombocytopenia or prothrombin deficiency
91
Antiphospholipid Ab syndrome - criteria - labs - tx
-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
What is the inheritance for all inherited thrombotic disorders - which one has the highest increase in risk? - which one is most common?
AUTOSOMAL DOMINANT ATIII deficiency -> highest risk (30x) Factor V leiden -> most common
93
Factor VIII:C inheritance vs. Factor VIII:Ag/vWF
Factor VIII:C inheritance -> sex-linked Factor VIII:Ag/vWF -> autosomal
94
MC inherited disorder of hemostasis?
von willebrand's disease followed by hemophilia A
95
MC bleeding manifestation of hemophilia?
Hemarthrosis
96
What does cryoprecipitate contain?
fibrinogen VIII vWF XIII
97
Which arch develops in common and internal carotid arteries?
arch 3 + part of dorsal aorta
98
right subclavian artery formed from? brachiocephalic? What happens to the right 6th arch?
4th arch + 7th intersegmental + intervening portion of dorsal aorta (RIGHT SIDE) right aortic sac -> brachiocephalic R 6th arch dissapears
99
Left side vessel embryo - aortic arch - left subclavian - ductus arteriosus/ligamentum arteriosum - pulmonary arteries
- 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
Arterial system - ventral - lateral - posterolateral
ventral -> digestive tract + glands lateral -> adrenal glands, gonads, kidneys posterolateral -> body wall
101
all 3 embryonic venous systems drain into the?
right/left horns of sinus venosus
102
Double SVC is due to?
Persistence of left anterior cardinal vein - will drain into R atrium by way of coronary sinus - problem is gets into the left atrium
103
2 causes of vascular rings
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
What's worse - preductal or postductal coarctation of the aorta?
Pre
105
What needs to be present for atrial and ventricular partitions to form?
Septum intermedium - formed by sup and inferior walls of endocardial cushion coming together
106
Aortic and semilunar valve formation
aortic valve -> right, left and posterior cusps Pulmonary -> right, left and anterior cusps
107
Pacemaker location in early development? S-A node A-v node
1. ventricle 2. right common cardinal or right sinus venosus 3. from superior endocardial cushion

Hematology-Oncology (3 decks)

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