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0001 STEP ONE RAPID REVIEW DX > Hem Onc > Flashcards

Hem Onc Flashcards

1
Q

Aplastic crises in sickle cell child

A

parvovirus b19

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2
Q

massive transfusions can cause hypocalcemia why?

A

whole blood and packed cells contain citrate anticoagulant which can chelate if given in large doses

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3
Q

hereditary index most specific for spherocytosis

A

elevation in mean corpuscular hemoglobin concentration

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4
Q

which region of the lymph node is populated by T lymphocytes and dendritic cells

A

paracortex of lymph node

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5
Q

region of lymph node poorly developed in digeorge

A

paracortex -

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6
Q

acute phase reactant synthesized by the liver that acts as a central regulator of iron homeostasis - influences body iron through interaction with ferroportin - transmembrane protein responsible for transferring intracellular iron into circulation

A

hepcidin - upon binding hepcidin, ferroportin is internalized and degraded, decreasing intestinal absorption and inhibiting the release of iron by macrophages (high iron levels thus would increase synthesis)

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7
Q

Transmigration of neutrophils out of the vasculature - i.e. squeezing between endothelial cells - is mediated by integrin attachments and adherence to

A

platelet endothelial cell adhesion molecule (PECAM-1) - this protein is found primarily at the peripheral intercellular junctions of endothelial cells

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8
Q

can lead to increased intracranial pressure in babies from not receiving appropiriate peri-natal care?

A

intracranial hemorrhage from vitamin K deficiency and thus ineffective clotting carboxylation

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9
Q

enzyme affected in acute intermittent porphyria

A

porphobiliongen deaminase (PBG deaminase)

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10
Q

enzyme targeted in in acute intermittent porphyria treatment?

A

ALA synthase - we want to suppress it! Give dextrose (inhibits PPAR-gamma, a TF for ALAsyn) and heme
avoid alcohol, smoking and p450 inducers

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11
Q

porphyria cutanea tarda - enzyme targeted

A

later in pathway of heme synthesis so don’s see toxic metabolite buildup causing neurologic symptoms -
uroporphyrinogen decarboxylase

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12
Q

porphyria cutanea tarda - signs

A

blistering cutaneous photosensitivity

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13
Q

The 5 Ps of Acute intermittent porphyria

A 
pain tummy
port wine pee
polyneuropathy
psych
precipitated by p450 inducers (and alcohol and starving)
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14
Q

What should you think of if you see increased 5-HIAA in 24 hour urine

A

carcinoid syndrome - this is a seratonin metabolite

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15
Q

surface marker of monocyte macrophage lineage

A

CD14 - seen in TB

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16
Q

EBV - which is associated with mono - which you see lymphocytes with abundant pale blue cytoplasm in a sea of red cells - also is known to cause (give me 4)

A

hodgkin
non hodgkin
burkitt
nasopharyngeal carcinoma

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17
Q

ondansetron
granisetron
dolasetron

A

highly effective in blocking chemotherapy induced vomiting via blocking central serotonin (5HT3) receptors in the area postrema (chemoreceptor trigger zone in 4th ventricle) and Nucleus tracuts solitarius (vomiting coordination center in medulla) - also located in the presynaptic nerve terminal of the vagus nerve in the GI tract

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18
Q

nucleus tractus solitarius

A

vomiting coordiation center in medulla

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19
Q

ruxolitnib

A

JAK2 inhibitor - rx for primary myelofibrosis (tear cell - massive splenomegaly)

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20
Q

what is bortezomib

A

a boronic acid containing dipeptide
proteosome inhibitor used for rx of multiple myeloma because ineffective proteosome fx leads to apoptosis and we want to kill the plasma cell that has gone bonkers

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21
Q

In addition to acting as an ADH (vasopressin) analogue, what other cool thing does desmopressin do?

A

it increases the release of vWF from endothelial cells - which augments platelet binding and also increases factor VIII stability - (note DDAVP also raises factor VIII)

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22
Q

What does hydroxyurea do / what is it used for

A

treatment of sickle cell disease - increases hemoglobin F synthesis

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23
Q

5Ps of AIP

A 
Painful abdomen
Port wine pee
Polyneuropathy
Pysch
Precipitated by drugs (cytochrome p450 inducers - phenobarbital, griseofulvin, phenytoin) alcohol, smoking, starvation
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24
Q

Rx iron poisoning

A

IV deferoxamine

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25
Q

PT tests

A

Extrinsic (i.e. TF / warfarin)

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26
Q

PTT tests

A

Intrinsic (all factors except VII and XIII - heparin)

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27
Q

Direct thrombin inhibitors (good to use if HIT)

3 examples

A

Argatroban
Hirudin
Dibigatran

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28
Q

Direct factor Xa inhibitors

A

Apixaban

Rivaroxaban

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29
Q

Danger in factor Xa inhibitors

A

No reversal agent if bleed

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30
Q

Reversal agent heparin bleeding?

A

Protamine sulfate

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31
Q

ADP receptor inhibitors

A 
Clopidogrel 
Pasugrel 
Ticagrelor - reversible
Ticlopidine 
Work because ADP required to stimulate GpIIbIIIa
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32
Q

Cilostazol

Dipyrdiamole

A

PDE-3 inhibitors

increased cAMP in platelets - leads to decreased aggrgation and increased vasodilation :)

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33
Q

GPIIbIIIa inhibitors

A

Abciximab
Eptifibatide
Tirofiban

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34
Q

Back pain in older person - sign it is likely malignancy?

A

WORSE AT NIGHT

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35
Q

Azathioprine (6-mercaptopurine) - mechanism

A

S (cc)
purine analog - decreases de-novo
activated by HGPRT - metabolized to 6MP

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36
Q

Azathioprine tox

A

myelosupression

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37
Q

Azathioprine (6-meracaptopurine) DD

A

metabolized by xanthine oxidase
xanthine oxidase is inhibited by allopurinol or febuxostat
must lower dose

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38
Q

cladribine (2-cda)

A

purine analog
S
also DS breaks

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39
Q

Cladribine use

A

Hairy cell

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40
Q

Cladribine - tox

A

myelosuppression

nephrotox

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41
Q

5-flurouracil - mech

A

pyrimidine analog

inhibits thymidylate synthase (complexes folic acid)

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42
Q

F-FU Tox

A

myelosuppression

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43
Q

Methotraxate

mech

A

folic acid analog

competitively inhibits DHF - decreased dTMP

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44
Q

MTX tox

A

myelosuppression
heptotoxic
mouth ulcers
pulmonary fibrosis

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45
Q

MTX tox reversible with?

A

Leucovorin (folinic acid)

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46
Q

Bleomycin - mech

A

G2

Induces free radical formation - break strands

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47
Q

Bleomycin tox

A

Pulmonary fibrosis
skin hyperpig
mucositis

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48
Q

Dactinomycin (actinomyccin D) - mech

A

intercalates DNA -

myelosuppression -children

49
Q

Doxorubicin mech

A

Generate free radical
intercalate DNA
DNA breaks

50
Q

Doxorubicin tox

A

cardio - dilated cardiomyopathy
myelosuppression
alopecia

51
Q

Doxorubicin - prevent toxicity with

A

dexrazoxane (iron chelator)

52
Q

busulfan - mech

A

cross link DNA

53
Q

Busulfan tox

A

myelosuppresion - severe
pulmonary fibrosis
pigment

54
Q

Cyclophosphamide - mech

A

cross link DNA at Guanin N7

require bioactivation in liver

55
Q

Cyclophosphamide - tox

A

myelosuppression -

hemorrhagic cystitis - give mesna

56
Q

Nitosureas (cramustine, lomustine, semustine, stretozocin) - mech

A

require bioactivation
cross blood brain barrier
cross link DNA

57
Q

Nitrosureas - tox

A

cns tox

convulsions, dizzy, ataxia

58
Q

carmustine

A

nitrosurea

59
Q

paclitaxel and other taxols - mech

A

hyperstabilize microtubules in M phase (ovarian and breast)

60
Q

paclitaxel - tox

A

myeslosuppression
alopecia
hypersensitivty

61
Q

vincristine - mech

A

vinca alakloid bind B tubulin - inhibit microtubule polymerization - solid tumors/leukemias/non-hodgkin

62
Q

vincristine - tox

A

neurotox

paralytic ilesu

63
Q

vinblastine - mech

A

same as vincristein - microtubule b blocker

64
Q

vinblastine tox

A

bone marrow suppression

neuro tox too?

65
Q

cisplatin, carboplatin

A

x link dna

66
Q

cispatin, carboplatin tox

A

nephro

oto

67
Q

cisplatin, carboplatin nephrotox prevetion

A

amifostine (free radical scavenger) chloride diuresis

68
Q

etoposide, teniposide - mech

A

topoisomerase II inhibitor - DNA degradation

69
Q

etoposide, teniposide - tox

A

myelosuppression, gi upset, alopecia

70
Q

irinotecan, topotecan - mech

A

topoisomerase I inhibitor -

71
Q

irinotecan, topotecan - tox

A

severe myelosupp, diarrhea

72
Q

hydroxyurea - mech (not for increasing HbF :)

A

inhibits ribonuclear reductase - decrases synthesis - S

73
Q

hydroxyurea - tox

A

myelosuppressoin

gi

74
Q

Bevacizumab

A

MAB against VEGF - inhibiting angiogenesis
solid tumors
hemorrhage - blood clots - impaired wound healing

75
Q

Erlotinib

A

EGFR TK inhibitor
NSC lung
Rash

76
Q

Imatinib

A

TK inhibitor for bcr-abl (CML) and c-kit (GI stromal)

fluid retention

77
Q

Rituximab

A

MAB against CD20 - b cells neoplasms

incrased risk of progressive multifocal leukoencephalopathy

78
Q

Tamoxifen, reloxifene

A

SERMS
block estrogen binding to ER+ cancer
note raloxifene also used for osteoperosis

79
Q

Tamoxifen tox

A

endometral cancer

80
Q

Trastuzumab

A

Herceptin - MAB Her-2 (TK receptor)

Cardiotoxicity

81
Q

Vemurafenib

A

small molecular inhibitor of BRAF - oncogene in melanoma

82
Q

cisplatin/carboplatin common tox

A

acoustic nerve damage

nephrotoxic

83
Q

vincristine common tox

A

peripheral neuropathy

84
Q

bleomycin (radical break dna) common tox

A

pulmonary fibrosis

85
Q

busulfan (cross link dna) common tox

A

pulmoray fibrosis

86
Q

trastuzumab - common tox

A

heart

87
Q

doxorubicin - common tox

A

heart

88
Q

cyclophsphamide - common tox

A

hemmorhagic cysitis - give mesna

89
Q

5-FU / 6-MP / Methotrexate common tox

A

myelosuppression .:(

90
Q

Important neutrophil chemotactiant

A 
IL8
LTB4
Kallikrein 
Platelet activating factor 
C5a
91
Q

Major basic protein

A

helminth toxin made by eos

92
Q

CD55/CD59

A

CD55 = decay accelerating factor
CD59 = MAC inhibitor
found in paroxysmal nocturnal hemoglobinuria -
error leads to MAC lack of protection in RBC - intravascuarl hemolysis

93
Q

burkitt lymphoma

A

t(8;14) - c-myc
EBV
Starry sky

94
Q

follicular lymphoma

A

t(14;18) - bcl-2
block apoptosis
wax and wane lymphadenopathy

95
Q

posterior auricular lymphadenopathy

A

rubella

96
Q

rubella -

A

togavirus - ssRNA (+)

97
Q

mantle cell lymphoma

A

t(11;14) - D-cyclin

CD5+

98
Q

Adult T-cell lymphoma

A

virus - HTLV - same retroviridae
lytic bone lesions
hypercalcemia

99
Q

Cutaneous t-cell lymphoma

A

Mycosis fungoides - odd skin CD4+ lesions

100
Q

Down syndrome

A

ALL association
Also
AML associateion

101
Q

CD10+

A

Marker of pre-B cells - good prognosis in ALL

t(12;21) also good

102
Q

Which leukemia is kids?

A

ALL

103
Q

Smudge Cells

A

CLL / SLL - characteristic cells

104
Q

Which leukemia can we see autoimmune hemolytic anemia

A

CLL - IgG - warm agglutinin - distinguish from lupus

105
Q

AUER RODS?

A

AML - t(15;17)
Rara
respond to trans-retinoic acid

106
Q

Common AML presenation

A

DIC

107
Q

Differentiate CML from Leukamoid reaction?

A

Low LAP in CML

108
Q

Rx AML

A

all trans retinoic acid

109
Q

Rx CML (if bcr;abl)

A

imatinib - small molecular inhibitor of TK

110
Q

Child comes in with ineffective T cell immune response and lytic bone lesions - what are you thinking? and what marker would you look for?

A

I am think Langerhans cell histiocytosis
S-100 and CD1a
Langerhans cells are functionally immature and do not effectively stimulate T cell proliferation
also see skin rash and otitis media with mass involving the mastoid bone

111
Q

common mutation in heamolytic myeloproliferative disorders?

A

JAK2

112
Q

Bone marrow contains large irregular megakaryocytes and you see jak2?

A

Essential thrombocytosis

113
Q

Tear drop RBCs

massive splenomegaly

A

Myelofibrosis - Jak2

obliteration of BM with fibrosis due to proliferation of monoclonal cell lines

114
Q

ALL myeloid cells are up?

A

Polycythemia vera

JAK2

115
Q

S-100 positive intracranial mass

A

schwannoma - look for biphasic patter of growth - marker?

116
Q

schwannomas can effect all but which cn?

A

II - only covered with oligodendrocytes

117
Q

schwannomas frequent effect which cranial nerve?

A

CVIII - acoustic neuroma
cerebellopontine angle
tennitis / vertigo / sensorinueral hearing loss

118
Q

most common variant of Hodgkin Lymphoma (think of this when poeple have B symptoms)

A

nodular sclerosis - which has a nodular growth pattern with surrounding fibrous bands and lacunar variant reed-sternburg cells

0001 STEP ONE RAPID REVIEW DX (25 decks)

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