heme-onc Flashcards

1
Q

what is the classic smear finding for myelofibrosis?

A

teardrop cells

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2
Q

what might you think of in young person with diarrhea and iron deficiency anemia?

A

celiac dz

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3
Q

how do you use methlymalonic acid and homocysteine in megaloblastic anemia workup?

A

If B12 is borderline (200-400) you can order
in folate def HC is up
in B12 def both are up

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4
Q

what biliary complication do patients with hereditary spherocytosis get?

A

cholelithiasis from bilirubin stones

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5
Q

how do you test for PNH? what are the major consequences of the disease?

A

flow for CD55 & CD 59
can p/w hemolysis, hemoglobinuria, Fe deficienc, arterial or venous thrombosis
(mechanism is genetic change that affects a surface protein that when missing makes cells susceptible to complement-mediated lysis)

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6
Q

true/false: steroids are effective for both warm (IgG) and cold( IgM) antibody-mediated autoimmune hemolytic anemia.

A

false – used for IgG only. for IgM, just keep person warm and tx underlying cause (neoplas or infxn (mycoplasma, EBV))

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7
Q

what might you suspect in someone who appears cyantotic with a normal O2 sat? causes? tx?

A

methemoglobinemia – o2 sat falsely high but PaO2 will be low
exposure to nitrites/nitrats, tylenol, dapsone, sulfas, ‘caines

tx with methylene blue

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8
Q

what is tx for methemoglobinemia?

A

methylene blue

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9
Q

give levels of transferrin saturation and ferritin for which people should be tested for hemochromatosis?

A

transferrin sat >45% (women) or >50% (men) or ferritin >1000

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10
Q

what bleeding problems do people with primary hemostasis problems (platelets) present with? what about secondary hemostasis (factor deficiencies)?

A

platelets - gingival bleeding, petechiae, echymoses

factors - hemarthrosis, hematomas (more deep tissue)

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11
Q

PT monitors which coag cascade? PTT?

A

PT – extrinsic
PTT - intrinsic

(PT is like physical therapy so it measure the “exercise” pathway)

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12
Q

what disorders might you think of in someone with a normal PT, prolonged PTT and bleeding problems?

what about the same pt but with clotting problems?

A

bleeding – Factor VII, Factor IX, Factor IX or Factor XII deficiency

clotting – antiphospholipid Ab syndrome

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13
Q

what is diagnosis in somene with clotting and prolonged PTT?

A

antiphospholipid Ab syndrome

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14
Q

compare/contrast ITP and TTP.

A

ITP: autoimmune, can be idiopathic or from drugs or infxn, tx with steroids or IVIG; splenectomy is 2nd line, rarely bleed, NO schistocytes

TTP: FAT-RN (fever, anemia, thrombocytop, ARF, neuro sx), get thrombosis, atx with plasmaphresis or steroids, + schistocytes

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15
Q

why do we avoid abx in kids who might have EHEC diarrhea?

A

giving abx increases the cahnce that they get HUS!

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16
Q

which two disorders that present with thrombocytopenia have a high clotting risk?

A

HIT and TTP – so never give platelets

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17
Q

T/F: THere are schistocytes in TTP but not in ITP.

A

true
ITP: autoimmune, no schistos, tx wtih steorids/IVIG
TTP: FAT-RN, get clots, +schistos

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18
Q

what are tx options for bleeding in factor VIII deficiency?

A

DDAVP (causes release of vWF and Factor VIII) or recombinant Factor VIII

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19
Q

how are factor VIII and Factor IX deficiences inherited? VWD?

A

factor VIII and IX are recessive

VMD is dominant

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20
Q

which factor deficiency results in a long PTT but NO clinical bleeding, even with surgeries.

A

Factor XII

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21
Q

what bleeding disorders can present with nl plt, PT, PTT and bleeding time?

A

mild VWD – can check levels of VWF and F VIII
mild hemophlia – can chek levels o F VIII and F IX
F XIII deficiency - can do “clot lysis” test (these peopl present with late post-surgical bleeding and poor wound healing)

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22
Q

why is warfarin initially pro-thrombotic?

A

proteins C and S decrease before factors bc they have shortest half-life

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23
Q

what is a common complication of patients iwth protein C deficiency who are started on warfarin?

A

warfarin skin necrosis

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24
Q

what should you test for in pts with warfarin-related skin necrosis?

A

protein C deficieny (predisposes)

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25
Q

what 3 antibpodies make up the antiphospholipid abs?

A

lupus anticoagulant Ab
anticardiolipin Ab
anti-beta2-glycoprotein 1 Ab

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26
Q

how does Factor V Leiden cause thrombosis? Do you have to be homozygote or heretero?

A

it causes resistance to activated protein C which normally acts to inactivate Factor V and VIII so with them unchecked you clot more

homozygotes have 20x risk of clotting, heteros 7x

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27
Q

what is tx for follicular lymphoma (usually diffuse LAD in middle-aged with +CD20) in asymptomatic pts? symptomatic?

A

asymptomatic – watchful waiting

symptomatic – rituximab, chemo and prednisone

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28
Q

what type of RBC transfusion should you give someone who has a h/o severe anaphylaxis with trasnfusion? what disease should you suspect in someone with a h/o severe anaphylaxis with transfusion?

A

washed (b/c they are reacting against hte plasma proteins?

IgA deficiency can p/w severe anaphylaxis to transfusion (bc people have anti-IgA Abs)

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29
Q

what immunoglobulin deficiency can p/w severe anaphylaxis to blood transfusion?

A

IgA deficiency

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30
Q

compare and contrast when you use tamoxifen, raloxifene and aromatase inhibitors in tx of hormone-receptor positive breast cancer.

A

premenopausal – use tamoxifen
postmenopausal – use aromatase inhib

raloxifene – only used for tx of osteoporosis

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31
Q

true/false asymptomatic pts with hairy cell leukemia should be treated.

A

yes – 1 round of cladribine cures >80%

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32
Q

what is treatment for hairy cell leukemia

A

cladribine

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33
Q

what leukemia might you suspect in an elderly man with pancytopenia, splenomegaly without lympadenopathy and a dry aspirate when BM is attempted? what is tx?

A

hairy cell leukemia

tx with cladribine even if asymptomatic b/c cures >80% of pts

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34
Q

what can you do tx hromone receptor positive breast cancer in a pre-menopausal pt with h/o DVT?

A

ovarian ablation (tamoxifen CI 2/2 clot history)

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35
Q

contrast/compare the electrophoresis findings for thalasemmic pts with alpha-thal trai and beta-thal minor.

A

alpha-thal trait: have nl electrophoresis results

beta-thal: elevated heglobni A2 band.

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36
Q

what is diagnosis of african-american woman with MCV 70, low RDW, hgb 11 wiht normal hgb electrophoresis?

A

alpha-thal trait

beta-thal would show elevated hgb A2 band

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37
Q

what is diagnosis of african-american woman with MCV 70, low RDW, hgb 11 with elevated hgb A2 band on electrophoresis?

A

beta-thal

alpha-thal trait would have nl hgb electrophoresis

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38
Q

give the three cytogenetic changes assoc with favorable prognosis in AML.

A

t(8;21) (M2)
t(15;17)(M3–APML)
inv16(M4)

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39
Q

give thee cytogenetic changes assoc with negative prognosis in AML.

A

inv(3)
del(5q)
del7

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40
Q
for each of the following cytogenetic characteristics of AML, say if they give a favorable or unfavorable prognosis:
inv(3)
t(8;21) 
t(15;17)
FLT3 mutation
del(5q)
del7
inv16
NPMI1 mutation
A

good: (t8;21),t(15;17), inv16, NPMI mutation
bad: del5q, del7, inv(3), FLT3 mutation

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41
Q

which AML is assoc with DIC?

A

APML (M3)

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42
Q

what is major SE of ATRA given to pts with APML?

A

ATRA sydnrome – fever, volume overload, effusions, respiratory distress and hypotension

tx by holding ATRA, giving steroids

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43
Q

what is standard induction for AML? who should be offered BMT?

A

ara-c x 7 days with daunorubicin x3 days

can tx if t achieve remission after induction or those with poor cytogenetics (inv3, del5q, del7)

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44
Q

what % of blasta on bone marrow defines acute leukemia?

A

> 20%

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45
Q

how is AML classified? ALL?

A

AML: M0 through M7 (M3 is APML)
ALL: precursor B, precursor T, mature B (Burkitt’s lyhphoma)

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46
Q

Is the t(9;22) translocation favorable or unfavorable in CML? In ALL?

A

CML: favorable
ALL: unfavorable

47
Q

what is typical chemotherapy for ALL?

A

prednisone, vincrstine, daunorubicin +/- cyclophosphamid + L-asaparginase

48
Q

is del5q favorable or unfavorable in AML? in MDS?

A

unfavorable in AML, favorable in MDS

49
Q

give two favorable cytogenetic markers for MDS.

A

del5q and del20q

50
Q

how do patients with del5q MDS present? what is tx?

A

p/w refractory anemia and thrombocytopenia

suusally respond to lenaliodmide

51
Q

what drug is used to treat CML with t(9;22)(BCR_ABL)? what are common SE?

A

imatinib

SE: cardiotoxicity, hepatotoxicity, cytopenia

52
Q

when do you treat essential thrombocythemia? with what?

A

tx if significant erythromelalgia symptoms (HA, visual changes, pain) with hydroxyurea

everyone (even asymptomatic) gets ASA to decreased clot risk

53
Q

is epo high or low in PV?

A

LOW! would be high in secondary causes

54
Q

is follicular lymphoma indolent or aggressive? what is classic cytologic marker? what is tx?

A

indolent
t(14;18) translocation which overexpresses BCL-2 (antiapoptotic protein)
can watch/wait if asympatomatic or

55
Q

T/F: diffuse large B cell that is present after transformation from follicular lymphoma is more aggressive than de novo dz.

A

true

56
Q

what cancer classically presents with a high white count and smudge cells on the smear?

A

CLL

57
Q

name 2 good prognostic indicators in CLL and 2 bad ones.

A

good: 13q del, low ZAP-70
bad: 17p del, 11q del

58
Q

what is standard tx for diffuse large B cell lymphoma?

A
R-CHOP
rituximab
cyclophosphamide
doxorubicin
vincristine
prednisone
if local dz, do 3 cycles + XRT
if widespread, 6-8 cycles

if recurs in young person, do autoSCT

59
Q

what cytogenetic dz is assoc with Burkett Lymphoma?

A

t(8;14)translocation, overexpresses C-MYC

60
Q

what lymphoma is t(14;18) assoc with? t(8;14)? t(11;14)?

A

t(14;18) –> BCL-2 –> Follicular
t(8;14) –> C-MYC –> Burkitts
t(11;14)–>BCL-1–>Mantle Cell

61
Q

what has a better prognosis, hodgkin or NHL?

A

hodgkin

62
Q

what lymphoma is assoc with Reed-Sternberg cells?

A

hodgkin lymphoma

63
Q

what is chemo for of hodgkin?

A
ABVD
adriamycin (doxorubicin)
bleomycin
vinblastine
dacarbazine
64
Q

what virus is associated with HIV-associated primary effusion lymphomaa?

A

HHV-8

65
Q

t/f: bone scan will show lytic lesions for multiple myeloma.

A

FALSE

MM lesions are lytic but do not show up on bone scan b/c no new bone being produced

do skeletal survey

66
Q

what is tx for plasmacytoma?

A

local XRT

67
Q

when do tx SVC syndrome emergently? how?

A

get tissue dx prior to tx unless increased ICP or airway compromise

tx with XRT (steroids controversial)

68
Q

what cancers can p/w lytic lesions?

A

MM, breast, renal

69
Q

t/f: biopsy any palpable non-cystic breast mass even if not seen on ultrasound

A

true – mammon on 80% sensitive

70
Q

what does erlotinib target?

A

EGFR

71
Q

what is tx for high-risk MDS (anemic, thrombocytopenic, 10-19% blasts)?

A

azacitadine

72
Q

how do you minimize risk of erythrocyte alloimmunization?

A

phenotypically match units (matches C, E and K Ag)

irrad prevents GVHD in IS, washing prevents allergic rxn

73
Q

t/f: washing RBC units reduces risk of alloimmunization.

A

false – lessens allergies

have to phenotypically match units (match C,E, K Ag) to reduce risk of alloimmunization

74
Q

what does Bevacizumab target? what are common SE?

A

targets VEGF

SE: HTN, vascular “catastrophes,” poor wound healing, intestinal perforation

75
Q

contrast/compare results of coombs test for:
warm agglutinin Ab
cold agglutinin Ab
hereditary spherocytosis

A

warm (IgG abx): IgG+, C3 neg
cold (IgM abx): IgG neg, C3+
spherocytosis: both neg

76
Q

what is tx for DCIS?

A

local excision with adequate margins +/- XRT (XRT reduces recurrence but doesn’t improve survival)

77
Q

is her2/neu a good or bad prognostic marker in breast cancer?

A

bad (altghou can use trastuzumab)

78
Q

what is trastuzumab against? what is its major SE?

A

her2/neu

cardiac toxicity

79
Q

true false - if do lumpectomy in breast cancer, you always do XRT.

A

true

80
Q

what is used for adjuvant hormonal tx in premenopausal women? postmenopausal

A

pre –> tamoxifen

post–> aromatase inhibi (anastrozole, letrozole, exemestane)

81
Q

t/f: pregnant women who have a history of idiopathic VTE should get enoxparin for ppx.

A

true

82
Q

T/F: you have to get a bx if you have LGSIL on pap smear

A

true

83
Q

which testicular cancer causes AFP elevation?

A

yolk sac (a nonseminomnous germ cell tumor)

84
Q

which testicular cancers can have elevated HCG?

A

choriocarcinoma – a nonseminous germ cell tumor – 100%

embyronal cell cancer – a nonseminomonous germ cell tumor – 50%

seminoma - germ cell tumor – 5-10%

85
Q

what testicular cancer do you suspect if someone has an elevated AFP?

A

yolk sac tumor

86
Q

describe staging for testicular cancers?

A

stage I - testis only
stage II - RP nodes
stage III - mets
stage IV - there isn’t one!

87
Q

what stage is testicular cancer involving RP nodes?

A

II

88
Q

what stage is metastatic testicular cancer?

A

III (there is no stage IV)

89
Q

describe classification of testicular cancers.

A

GERM CELL (95%) or STROMAL (5%)
stromal: leydig or sertoli
GC: seminoma (good prognosis) or nonseminoma

nonseminoma: embryonal, teratoma, choriocarcinoma (HCG+), yolk sac (AFP+)

90
Q

T/F: Metastatic seminoma of the testicle is curable.

What is tx?

A

true

orchiectomy
XRT to RP nodes
if nodes >5 cm or mets –> chemo

91
Q

describe mgmt of patients with:

T1-T2N0M0 prostate CA
T1-T3N0M0 prostate CA
prostate CA wtih high (7-10) Gleason score

A

T1-T2: radical prostatectomy
T1-T3: XRT
high Gleason: hormonal deprivation tx

92
Q

hwo do you tx a woman with adenocarcinoma of unknown primary in axillary nodes?

A

like stage II breast CA – mastectomy

93
Q

what is tx for localized carcinoid tumor? metastatic?

A

localized – resection

metastatic - symptomatic tx

94
Q

whihc MEN syndrome includes carcinoid tumors?

A

MENI

95
Q

what does imatinib bind? what cancers is it used in?

A

binds tyrosine kinase preventing cell signaling

used in CML and GIST tumors

96
Q

what does traztuzumab bind? SE to know?

A

anti HER2/neu

can cause reversible CHF

97
Q

what chemotx is assoc iwth hemorrhagic cystitis?

A

cyclophosphamide

98
Q

what are major toxicities of platinums (cisplatin, carboplatin, oxalitplatin

A

oto/neurotoxic
nephrotoxic
myelopsuppressive

99
Q

what is major SE of busulfan?

A

interstitial pneumonitis/pulm fibrosis

100
Q

what are the following monoclonals active against:

  • bevacizumab
  • cetuximab
  • erlotinib
A

bevacizumab: VEGFR

cetuximab/erlotinib: EGFR

101
Q

what is major SE of bleomycin?

A

irresversible pulmonary fibrosis

102
Q

what chemo causes magnesium-wasting?

A

cisplatin

103
Q

describe how SCLC is staged and treated.

A

limited (1 XRT field) - chemo + chest XRT + brain XRT

extended - chemo only

104
Q

T/F: Kidney cancer is the only malignancy in which removing the primary tumor in the setting of metastatic disease can improve overall outcome rather than just reduce local symptoms.

A

true!

105
Q

t/f: if a breast cancer has a bony met, you need to bx it.

A

yes! b/c hormone and Her2 statuses are sometimes diff in bony mets so tx can change

106
Q

what is the platelet goal for transfusion for major surgeries? CNS surgeries?

A

CNS is 100K

everything else 50K

107
Q

what do pts with alloAb get with transfusion? IgA deficiency

A

IgA deficiency –> anaphylaxis (they have anti-IgA ab that activate against donor serum)

alloAb (freq transfusion recipients) –> 5-10 days later get jaundice, hemolytic anemia

108
Q

how do you screen for hereditary angioedema? diagnose?

A

screen: look for low C4 level
dx: check C1-INH functional assay

109
Q

true/false. herediatry angioedema, unlikely angioedema from an immediate hypersensitivity reaction, does NOT cause itching or urticaria.

A

true!

110
Q

what infection do patients with the following complement deficiencies get?:

  • C1, C2 or C4
  • C3
  • C5-C9
A

C1,2,4: recurrent sinopulmonary infxns, increased early-onset rheum dzs
C3: severe pyogenic bacterial infxns
C5-C9: increased neisseria infxns

111
Q

deficiency of which complement is assoc with neisserial infxns/

A

C5:C9 (diagnse by checkign CH50)

112
Q

deficiency of which complement is assoc with severe pyogenic bacterial infxns?

A

C3

113
Q

what infxns do people with congenital agammaglobulinemia get?

A

pyogenic/encapsulated organisms
so recurrent sinopulmonary infxns

nl protection against fungi
do okay against most GNs and viruses (have T cell)

114
Q

what presents with eczema, low IgM, high IgA and IgE and thrombocytopenia? tx?

A

Wiskott-Aldrich (Wisk through the EXIT (eosinophilia, X-lined, immunodeficiency, thrombocytopenia)

tx wth BMT