Heme/Onc Flashcards

(70 cards)

1
Q

Virchow’s triad

A
  1. Vessel injury
  2. venous stasis
  3. hypercoagulability
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2
Q

Top 5 hereditary RFs for thrombophilia

A
  1. Factor V Leiden
  2. Prothrombin mutation
  3. Protein C deficiency
  4. Protein S deficiency
  5. Antithrombin deficiency
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3
Q

Top 5 acquired RFs for thrombophilia (RR)

A
  1. Antiphospholipid Abs (10)
  2. Active cancer (7)
  3. Pregnancy (6)
  4. Estrogen (OCPs/HRT) (4)
  5. Smoking
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4
Q

Multiple RFs (either hereditary or acquired) have a additive/synergistic effect on RR of first VTE event?

A

synergistic

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5
Q

What clinical AND laboratory info is necessary to make a dx of Antiphospholipid syndrome?

A
  • Clinical criteria: arterial/venous thrombosis OR pregnancy loss/ recurrent miscarriage
  • Labs criteria: (+) APLA test (any of the 3) detected on 2 or occasions separated by at least 12 weeks
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6
Q

Why do you need a (+) APLA test (any of the 3) detected on 2 or occasions separated by at least 12 weeks?

A

can be falsely elevated d/t dz

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7
Q

MC inherited thrombophilia

A

Factor V Leiden

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8
Q

Homozygous neonates may present clinically with neonatal purpura fulminans (DIC) w/ these 2 hereditary thrombosis RFs:

A
  1. Protein C deficiency
  2. Protein S deficiency (cofactor for APC)
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9
Q

What effect does warfarin have on activated protein C (APC)? Why?

A

↓ levels because protein C is dependent on Vitamin K and warfarin is a Vitamin K antagonist

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10
Q

Name the dz based on clinical presentation:

  • VTE (may be recurrent) @ unusual sites
  • Recurrent thrombosis may occur despite absence of additional RFs
  • Some patients are resistant to heparin therapy
A

antithrombin deficiency

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11
Q

How does the presence of APLAs mess with the following coagulation labs: aPTT, PT, 1:1 mixing study

A
  • aPTT: prolongs aPTT → makes heparin dosing difficult
  • PT: may prolong PT → makes warfarin dosing difficult
  • 1:1 mix: does NOT correct BUT causes CLOTTING not bleeding (unlike clotting factor deficiency disorders)!!
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12
Q

Do APLAs act as anti- or pro- coagulants?

A

PROCOAGULANTS!! 1:1 mixing study does NOT correct but causes CLOTTING not bleeding!

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13
Q

What is the initial therapy for tx of VTE (2 components)?

A
  • heparin or LMWH for ~5 days
  • Warfarin/coumadin for 3-6 mo.
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14
Q

When should you test for abnormal hypercoagulable states in the clinic?

A

When results could change management!

  • Young age (<45 yo) w/ unprovoked VTE questions regarding need for long term anti-coagulation therapy
  • Thrombosis in unusual sites (arterial, upper extremities)
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15
Q

What are the 3 new anti-coagulation drugs that may change the risk/benefit analysis that is currently an issue with warfarin? What is one potential problem with these drugs?

A
  • Dabigatran (Pradaxa)
  • Rivaroxaban (Xarelto)
  • Apixaban (Eliquis)

NO OD REVERSAL AGENT!!

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16
Q

What are the 3 APLAs?

A
  1. Lupus anticoagulant
  2. Anticardiolipin Abs
  3. anti-β2GPI Abs
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17
Q

What is the biggest thrombophilia RF contributing to recurrent clotting events

A

persistent APLAs

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18
Q

What are 4 modifiable RFs that can be addressed during tx of thrombophilia?

A
  1. smoking cessation
  2. maintaining healthy weight
  3. staying active (lack of immobilization)
  4. avoiding estrogen/ progesterone
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19
Q

Which 2 hereditary VTE RFs likely need additional RFs in order to provoke thrombosis?

A
  • Protein C deficiency
  • Protein S deficiency
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20
Q

What are 3 lab tests we have to test platelet function?

A
  • PFA-100
  • Platelet aggregation testing
  • Thromboelastography
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21
Q

What is the most important lab test for differentiating b/w a coagulation factor deficiency and a coagulation factor inhibitor?

A

1:1 mixing study

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22
Q

Does a 1:1 mix study correct if a coagulation factor deficiency or inhibitor is present?

A

deficiency: 1:1 mix corrects to normal

inhibitor: 1:1 mix does NOT correct to normal

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23
Q

leading cause of renal failure in children worldwide

A

HUS d/t shiga toxin in E. Coli

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24
Q

What are the 3 requirements for proper RBC prdn. (erythropoiesis)?

A
  1. bone marrow cells working
  2. EPO from kidney
  3. substrate supplies (iron, Vit. B12, folic acid)
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25
define anemia:
low Hb or Hct Hct = Hb x 3
26
The severity of anemia ssx depends on what?
speed at which the anemia developed
27
What does a reticulocyte index (RI) \< 2.0 indicate?
inadequate BM response to anemia
28
What is iron-deficiency testing confounded by in many anemic patients?
inflammation --\> ferritin ↑ as an acute-phase reactant → mask low level of ferritin expected in Fe deficiency
29
Does Vit. B12 or Folate deficiency typically cause neuro ssx w/ (+) hx of AI dz?
B12 deficincy
30
↑↑ levels of methylmalonate AND homocysteine suggest which type of macrocytic anemia?
B12 deficiency
31
MCC of anemia in the western world
Fe-deficiency
32
RBC smear: hypochromia (pale RBCs) + microcytosis (small RBCs) + anisocytosis (RBCs of different sizes)
Fe deficiency anemia (late)
33
Which racial population is α-thalassemia MC in?
SE asians
34
Which racial populations is β-thalassemia MC in?
Mediterranean and African | (but has world-wide distribution)
35
Single nucleotide substitution in β-globin gene on chromosome 11
sickle cell dz
36
hallmark of sickle cell dz
pain crisis
37
What tx do β-thalassemia major patients need regularly?
RBC transfusions
38
RBC smear: Rouleaux formation
multiple myeloma causing myelodysplastic syndrome
39
What Hct and Hb levels for men and wome indicate erythrocytosis (polycythemia)?
* women: Hct \> 48, Hb \> 16.5 * men: Hct \> 52, Hb \> 18.5
40
Tx for polycythemia vera
phlebotomy
41
labs: ↓Fe ↓TIBC (transferrin) ↑ferritin
anemia of chronic dz inflammation → ↓ Fe transport (transferrin) → ↓Fe release from m∅s
42
What does transferrin do (Fe metabolism)?
**trans**ferring **trans**ports Fe in the blood TIBC is an indirect measure of transferrin
43
What does ferritin do (Fe metabolism)?
1° Fe storage protein of the body
44
labs: ↓Fe ↑TIBC (transferrin) ↓ferritin ↓↓ % transferrin saturation (serum Fe/ TIBC)
Fe deficiency anemia
45
What are the 3 types of venous dz we learned about?
* chronic venous insufficiency (stasis) * DVT * Lymphedema
46
spider/ varicose veins
chronic venous insufficiency
47
2 Dx tests of chronic venous insufficiency; which is the most sensitive and specific?
Duplex ultrasound DVT (most sensitive and specific)
48
In what situation would you use a CT Scan/ MRI to dx chronic venous insufficiency?
May-Thurner Syndrome
49
Management of chronic venous insufficiency:
* Compression stockings * Leg elevation * then surgical options
50
Gold standard surgical tx of chronic venous insufficiency
saphenous vein ablation
51
Major complication of DVT requiring catheter directed thrombolysis
phlegmasia * Cerulea (cyanotic) Dolens- cancer (40%) * Alba (white) Dolens
52
What is reperfusion syndrome?
release of inflammatory mediators upon blood flow restoration in DVT 30-40% mortality in ICUs
53
Cause of chronic DVT (post-thrombotic syndrome)
venous valve dysfunction → chronic leg pain, eczema, swelling, venous ulcers
54
What is lymphedema?
accumulation of interstitial fluid d/t defective lymphatic drainage
55
Comme surgery often leading to lymphedema
breast mastectomy
56
PE: * edema * square toes * Stemmer's sign: inability fo pinch the skin over the dorsum of the 2nd toe
lymphedema
57
mainstay tx for lymphedema
compression stockings
58
blood smear: PMNs w/ toxic granulations + Dohle bodies
'toxic' PMNs → indicative of infectious (reactive) neutrophilia
59
absolute neutrophil count \< 1000
neutropenic fever → hematologic emergency
60
Why is neutropenic fever a hematologic emergency? Tx?
d/t high risk of gram(+) bacteremia Tx: empirically tx w/ broad coverage abx
61
dx pathology: \> 20% blasts in periphery + Auer rods
AML
62
t(9:22) bcr-abl
CML
63
peripheral blood smear: smudge cells
SLL/CLL
64
What can prolong Prothrombin Time (PT)? (3)
* liver dz * Vitamin K deficiency * Warfarin\*\*
65
PT/INR is most sensitive to what coagulation factor?
VII (extrinsic pthwy)
66
PTT is most sensitive to which coagulation factors?
VIII and IX
67
MC lymphoma
Diffuse B cell lymphoma (aggressive NHL)
68
Reed-Sternberg ('owl eye') cells
Hodgkins Lymphoma
69
common ssx for this lymphoma is pruitis (itchyness) w/o rash
Hodgkins
70