Heme/Onc Flashcards

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
Q

define anemia:

A

low Hb or Hct

Hct = Hb x 3

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26
Q

The severity of anemia ssx depends on what?

A

speed at which the anemia developed

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27
Q

What does a reticulocyte index (RI) < 2.0 indicate?

A

inadequate BM response to anemia

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28
Q

What is iron-deficiency testing confounded by in many anemic patients?

A

inflammation –> ferritin ↑ as an acute-phase reactant → mask low level of ferritin expected in Fe deficiency

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29
Q

Does Vit. B12 or Folate deficiency typically cause neuro ssx w/ (+) hx of AI dz?

A

B12 deficincy

30
Q

↑↑ levels of methylmalonate AND homocysteine suggest which type of macrocytic anemia?

A

B12 deficiency

31
Q

MCC of anemia in the western world

A

Fe-deficiency

32
Q

RBC smear:

hypochromia (pale RBCs)
+ microcytosis (small RBCs) + anisocytosis (RBCs of different sizes)

A

Fe deficiency anemia (late)

33
Q

Which racial population is α-thalassemia MC in?

A

SE asians

34
Q

Which racial populations is β-thalassemia MC in?

A

Mediterranean and African

(but has world-wide distribution)

35
Q

Single nucleotide substitution in β-globin gene on chromosome 11

A

sickle cell dz

36
Q

hallmark of sickle cell dz

A

pain crisis

37
Q

What tx do β-thalassemia major patients need regularly?

A

RBC transfusions

38
Q

RBC smear:

Rouleaux formation

A

multiple myeloma causing myelodysplastic syndrome

39
Q

What Hct and Hb levels for men and wome indicate erythrocytosis (polycythemia)?

A
  • women: Hct > 48, Hb > 16.5
  • men: Hct > 52, Hb > 18.5
40
Q

Tx for polycythemia vera

A

phlebotomy

41
Q

labs: ↓Fe ↓TIBC (transferrin) ↑ferritin

A

anemia of chronic dz

inflammation → ↓ Fe transport (transferrin) → ↓Fe release from m∅s

42
Q

What does transferrin do (Fe metabolism)?

A

transferring transports Fe in the blood

TIBC is an indirect measure of transferrin

43
Q

What does ferritin do (Fe metabolism)?

A

1° Fe storage protein of the body

44
Q

labs: ↓Fe ↑TIBC (transferrin) ↓ferritin ↓↓ % transferrin saturation (serum Fe/ TIBC)

A

Fe deficiency anemia

45
Q

What are the 3 types of venous dz we learned about?

A
  • chronic venous insufficiency (stasis)
  • DVT
  • Lymphedema
46
Q

spider/ varicose veins

A

chronic venous insufficiency

47
Q

2 Dx tests of chronic venous insufficiency; which is the most sensitive and specific?

A

Duplex ultrasound

DVT (most sensitive and specific)

48
Q

In what situation would you use a CT Scan/ MRI to dx chronic venous insufficiency?

A

May-Thurner Syndrome

49
Q

Management of chronic venous insufficiency:

A
  • Compression stockings
  • Leg elevation
  • then surgical options
50
Q

Gold standard surgical tx of chronic venous insufficiency

A

saphenous vein ablation

51
Q

Major complication of DVT requiring catheter directed thrombolysis

A

phlegmasia

  • Cerulea (cyanotic) Dolens- cancer (40%)
  • Alba (white) Dolens
52
Q

What is reperfusion syndrome?

A

release of inflammatory mediators upon blood flow restoration in DVT

30-40% mortality in ICUs

53
Q

Cause of chronic DVT (post-thrombotic syndrome)

A

venous valve dysfunction → chronic leg pain, eczema, swelling, venous ulcers

54
Q

What is lymphedema?

A

accumulation of interstitial fluid d/t defective lymphatic drainage

55
Q

Comme surgery often leading to lymphedema

A

breast mastectomy

56
Q

PE:

  • edema
  • square toes
    • Stemmer’s sign: inability fo pinch the skin over the dorsum of the 2nd toe
A

lymphedema

57
Q

mainstay tx for lymphedema

A

compression stockings

58
Q

blood smear:

PMNs w/ toxic granulations + Dohle bodies

A

‘toxic’ PMNs → indicative of infectious (reactive) neutrophilia

59
Q

absolute neutrophil count < 1000

A

neutropenic fever → hematologic emergency

60
Q

Why is neutropenic fever a hematologic emergency? Tx?

A

d/t high risk of gram(+) bacteremia

Tx: empirically tx w/ broad coverage abx

61
Q

dx pathology:

> 20% blasts in periphery + Auer rods

A

AML

62
Q

t(9:22) bcr-abl

A

CML

63
Q

peripheral blood smear: smudge cells

A

SLL/CLL

64
Q

What can prolong Prothrombin Time (PT)? (3)

A
  • liver dz
  • Vitamin K deficiency
  • Warfarin**
65
Q

PT/INR is most sensitive to what coagulation factor?

A

VII (extrinsic pthwy)

66
Q

PTT is most sensitive to which coagulation factors?

A

VIII and IX

67
Q

MC lymphoma

A

Diffuse B cell lymphoma (aggressive NHL)

68
Q

Reed-Sternberg (‘owl eye’) cells

A

Hodgkins Lymphoma

69
Q

common ssx for this lymphoma is pruitis (itchyness) w/o rash

A

Hodgkins

70
Q
A