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Haem/Onc > AML, ASPHO > Flashcards

AML, ASPHO Flashcards

1
Q

what are the BIG FOUR cytogen anomalies in AML?

A

t(8;21)= AML1-ETO;
inv (16)/t16;16) CBFB MYH11;
t(15;17) PML-RARA, variants
abnormal 11q23 MLL-partner

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2
Q

characteristics of AML with t(8;21)?:

A

Auer rods, chloromas, good prognosis

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3
Q

characteristics of AML with inv(16)/t(16;16)?

A

Eos with baso granules, chloromas, good prog

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4
Q

characteristics of AML with (15;17)?

A

granules/auer rods, DIC, bleeding, good prog with ATRA/arsenic

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5
Q

characteristics of AML with 11q23?

A

infant WBC/skin/CNS/gums t-AML after top II inh

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6
Q

in APML, cells blocked where during dev?

A

promeylocyte

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7
Q

what does ATRA do in AML?

A

overcomes the “differentiation block” at the promyelocyte stage

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8
Q

Give 5 major myeloid luekemia predispositions

A

Germline mutations in CEBPA, DDX41, RUNX1, ANKRD26, ETV6, GATA2…also bone marrow failure syndromes (fanconi anemia, Dyskeratosis congenita), T21, NF1 (JMML), Noonan (JMML)

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9
Q

2 marrow failure syndromes–> pancytopenia taht are associated with MDS/AML

A

Fanconi anemia, Dyskeratosis congenita

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10
Q

marrow failure syndrome–> anemia associated with MDS?

A

Diamond Blackfan Anemia

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11
Q

marrow failure syndrome–> neutropenia associated with MDS?

A

Schwachman-Diamond, Kostmann Syndrome

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12
Q

marrow failure syndrome–> thrombocytoepnia associated with MDS/AML?

A

congenital amegakaryocytic thrombocytopenia (MPL mutation)

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13
Q

environemntal causes of AML?

A

alkylating agents, radiation, toposiomerase II inhibitors (etoposide> anthracyclines)

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14
Q

What is the difference in latency between AML caused by different environemntal agents?

A

alkylating agents and radiation: 5-7 years from exposure, common preceding MDS

topoisomerase II inhibitors: short latency (1-2 years) from exposure, rare preceding MDS

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15
Q

give the diff in cytogenetics between AML caused by different envrionemntal agents?

A

alkylating agents and radiation: monosomy 7, 7q deletions, monosomy 5, 5q deletions

topoisomerase II inhibitors: 11q23 rearrangement most common, but t(8;21), t(15;17), t(9;22), and inv(16) can also occur

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16
Q

what tends to be more refractory to treatment? treatment-related AML/MDS or de novo AML/MDS?

A

treatment-related

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17
Q

AML and CML are most common in what age group?

A

> 50 years

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18
Q

concordance rate of AML/ALL in identical twins= monozygotic?

A

depends on age of first twin when diagnosed…<1 year: close to 100% concordance (suggests prenatal preleuekmic event= KMT2A and intrauterine transfer)….age 1-5: 10-20% concordance, most = clonal, longer-latency between twins A and B…likely a prenatal preleuk event (ETV6-RUNX1, RUNX1T1, eg, intrauterine transfer); relatively lower risk of cooperating hits (unlike KMT2A)….case 6+: minimal increased risk

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19
Q

concordance for ALL/AML in fraternal twins?

A

minimal increased risk

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20
Q

In AML, is age used to risk stratify? gender? race? BMI? pharmacogenomics? high WBC?

A

No for all…except WBC>10k is high risk in APL–> use anthracycline or gemtuzumab immediately….but note that non-whites have inferior survival, under-BMI and over-BMI have inferior survival as well

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21
Q

Give 4 favourable risk stratifiers in AML

A

T21 (esp if <4); APL with t(15;17); inv (16) or t(8;21); NPM1 or CEBPA mutations

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22
Q

Give 4 unfavourable risk stratifiers in AML

A

treatment-related AML, MDS-related AML, monosomy 7, 5 q deletion, abnormal 3q, FT3/ITD especially if high allelic ratio (0.4+), mutation, primary induction failure =>5% blasts after course 2

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23
Q

Intermediate risk AML can be subclassified into favourable or unfavourable how?

A

MRD by flow cytometry after first block of therapy…using threshold of 0.1%

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24
Q

describe M0 AML

A

“aml without differentiation”…not differentiated, no MPO expressed

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25
Q

describe M2 AML

A

AML with differenitation…8;21 translocation, lots of auer rods, chloromas, good prog

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26
Q

describe M3 AML

A

“APL= acute promyelocytic leukemia, hyergranular variant”, t(15;17), bleeding/DIC

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27
Q

describe M3v AML

A

APL, microgranular variant of M3

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28
Q

Describe M1 AML

A

“AML with minimal differentiation”…minimal diff, MPO detectable

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29
Q

describe M4 AML

A

“acute myelomonocytic AML”…myeloblasts + monoyctic blasts…peripheral monos too

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30
Q

Describe M4Eo

A

“AMML with eosinophilia”..abnormal eos precursors in marrow, inv(16)

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31
Q

M5 AML?

A

“acute monocytic leukemia”…MLL11q23! CNS involvement, chloromas, gingival hyperplasia

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32
Q

M6 AML?

A

“acute erythroblastic leuk”..rare in kids

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33
Q

M7 AML?

A

“acute megakaryoblastic leuk”…T21, gata 1 mutations, myelofibrosis common

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34
Q

WHO classification requires how many blasts?

A

20% blasts in marrow (unlike 30% in FAB)

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35
Q

which AML Dx’s can be made by yes/no qs on the WHO criteria?

A

Therapy-related AML= t-AML; DS-related AML, AML with abnormality (if recurreing molecular abnormality present, regardless of blast count), AML with MDS-related changes (if dysplasia, prior MDS and/or MDS-related mutation= monosomy 7, del 5q, etc)…if no to all, AML NOS and use FAB to subclassify

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36
Q

what is myeloid sarcoma

A

chloroma…can be manifestation of AML for any AML dx

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37
Q

give 6 mutations seen in AML

A 
t(8;21)
inv(16)
t(9;11) MLLT3-KMT21
t(6;9) DEK-NUP214
inv(3) q3 mutations; t(3;3) gata2, MECOM
megakaryblasts with t(1;22) RBM15-MKL1
mutated NPM1
biallelic mutations of CEBPA
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38
Q

give 3 pan-myeloid markers…which FAB type is an exception?

A

MPO, CD13, CD33…but not in M7 (no MPO, rare CD13)

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39
Q

what marker is specific to M7?

A

CD41a!!!!! 42, 61, no MPO!

40
Q

which markers are specific to monocytic AML M4, 5

A

CD14! CD11b

41
Q

which markers are specific to erythrocytic AML M6

A

glycophorin A= cd235a

42
Q

APL markers? (M3)

A
  • these cells AUTO-FLUORESCE…high on the diff axes even without diff Abs
  • CD33 (think gemtuzumab), HLA-DR negative, CD34 neg
43
Q

t(8;21) AML marker?

A

can often co-express CD19+

44
Q

what is the RAM immunophenoytpe?

A

flow pattern characterized by bright CD56, min CD45, min CD38, neg HLA-DR–> poor prognosis

45
Q

acute luekemia of ambigous lineage: 2 types?

A
  • acute undifferenatied leukemia

- mixed phenotype acute leukemia

46
Q

what do you see in acute undifferenatied leukemia?

A

no lineage-specific markers

47
Q

what do you see in mixed phenotype acute leukemia?

A

-distinct populations of blasts from diff lineages and/or sngle opulation of blasts co-expressing antigens of multiple lineages

48
Q

mixed phenotype acute leuekmia are often what?

A

MLL-rearranged (“mixed lineage leukemia); t/myeloid, b/myeloid most common mixtures

49
Q

current standard of care for AML, first part?

A

-Remission induction = two 4-week courses of intense chemo: Doxo and Ara-C and Etop, 10 days for first course, 8 days for second course; gemtuzumab= anti-CD 33 immunotoxin for first course

50
Q

what’s concernign about first part of AML tx?

A
  • high risk of invasive infection

- only 75-80% go into complete remission

51
Q

post-remission consolidation invovles what?

A

two ways: transplant or go to more chemo

  • transplant all high risk patients with best available donor…don’t transplant the LR…typically occurs after 1 chemo consoliation course with high dose Ara-C (3 courses total)
  • additional chemo intesnifiation courses 2-3, for any HR pts without any donor, and all LR pts…2 courses sufficient for LR with low MRD but 3 courses best for everyone else…usually give high dose Ara-C combine with drugs not used in induction like mitoxantrone, L-asp
52
Q

role of CNS ppx in AML?

A
  • much less intense than in ALL due to low rates of CNS realpse in AML
  • if CNS + at dx, give weekly IT triples= MTx, Ara-C, HC) until CSF clears, then once per course
53
Q

do you give cranial radiation in AML?

A

no

54
Q

is there a role of maintenance chemo in AML?

A

no (excpet APML)

55
Q

what’s the new guideline re: LP for AML?

A

wait until day 8 for LP to avoid contamination wtih peripheral blasts

56
Q

AML patients should be admitted during periods of ?

A

neutropenia

57
Q

bacterial infections that we worry about in AML?

A

strep viridans, gram neg enterics: E. coli, pseudomonas, klebsiella

58
Q

what type of ppx is used in AML?

A
  • bacterial ppx controversial
  • septra for PCP
  • fungal ppx used (fluco?)
  • dexrazoxane for heart protection
59
Q

use GCSF in AML?

A

no, due to concern re: promoting leukemia cell growth/survival

60
Q

m3 AML= APML..high risk of what? do waht?

A

life-threatening bleeds due to DIC, fibrinolyisis + low plts…close monitoring of INR, PTT, fibirngeon, d-dimer, plts with liberal use of plts, cryo, FFP…give ATRA ASAP!

61
Q

what are the features of differention syndrome? 5

A 
Fever
edema
pulm infiltrates
hypoxia
resp distress
hypotension
renal dysufnction
hepatic dysufnction
effusions 
rash
62
Q

higher risk of differentiation if what is present?

A

high WBC

63
Q

tx differnetion syndrome how?

A

dexmathesone; hold ATRA until resolved

64
Q

tx for APML other than ATRA?…benefit?

A

arsenic trioxide, helps reduce anthra exposure

65
Q

diff between AML in kids and audlts?

A

in adults, usually starts iwth MDS

66
Q

tx for MDS?

A

SCT

67
Q

hyperluekocytosis=?

A

WBC>100k

68
Q

hyperleuk more common in AML or ALL?

A

AML

69
Q

organs affected in hyperleuk?

A

brain, lungs, kidney

70
Q

manage hyperleuk how?

A
  • treat leuk asap

- exhcnage trasnfusion vs. leukopheresis if symptoms

71
Q

3 major late effects in AML?

A
  • cardiomyopathy due to anthracyclins…doxo 450 mg/m2
  • translplant-related MDS/AML from etoposide
  • neurotox from high dose Ara-C
72
Q

2 ways to reduce cardiotox

A

dexrazoxane, ACE inhibitors

73
Q

indications for radiation in AML?

A
  • emergent treatment for life-threatneing tx of chloromas, like spinal cord compression
  • TBI is NOT a typical component of conditioning for SCT in AML
74
Q

mutation in CML?

A

t(9;22) BCR-ABL1

75
Q

3 phases of CML?

A

chronic phase (<10% blasts in marrow and blood)

  • accelerated phase (10-19% in marrow or blood of at least 20% basophilis in blood)
  • blast crisis= acute leukemia, either lymphoid or myeloid, at least 20% blasts in marrow
76
Q

most CML patients diagnosed in which pahse?

A

chronic

77
Q

CBC findings in CML at dx?

A
  • high WBC, left shift
  • may have anemia
  • may have high plts
  • basophilia
  • may have eosinophilia
78
Q

symptoms in CML?

A
  • abdo pain
  • dysphagia
  • increased abdo girth, splenomegaly
  • fatigue
  • wt loss
  • night sweats
  • bleeding (rarely) because high plts–> acquired VWD
  • leukostasis/priapsim
79
Q

CML: blast crisis more likely to be AML or ALL?

A

AML in 2/3, ALL in 1/3

80
Q

p190 bcr-abl fusion in?

A

pH+ ALL

81
Q

p210 in?

A

CML

82
Q

do what for recurrent or refractory chornic phase CML?

A

-try higher dose TKI and/or new TKI, then SCT if tx failure

83
Q

for accelerated phase or blast crisis, do what?

A

TKI to get them back into chronic phase, then take them to transplant

84
Q

1st gen TKI?

A

imatinib

85
Q

2nd gen TKI?

A

Dasatinib, Nilotinib

86
Q

newer TKI?

A

Ponatinib…active against T315I mutation

87
Q

proven cure for CML?

A

SCT

88
Q

major prog factor for SCT?

A

disease phase! CP>AP>BC

89
Q

CML is VERY sensitive to waht? do what if relapse after HSCT?

A

graft vs. leukemia effect! …give DLI! especially if no GVH with first HST

90
Q

when HSCT is used, typically do what after?

A

TKI maintenance to prevent relapse

91
Q

3 ways to assess CML repsonse to tx?

A
  • HEME response: normal counts, no HSM
  • CYTOGENETIC response: bcr-abl fish on BMA…complete = no Ph+ vs. partial
  • MOLECULAR: BCR-ABL qPCR on peripoheral blood…complete= PCR neg…major= bcr-abl: abl ratio <0.1% or 3 log reduction in copy #…this is different from complete molec response = 4.5 log or 0.0032%
92
Q

do you need to do FISH BMA for CML if you can do pcr on peripheral blood?

A

no

93
Q

check CML response how often?

A

q3 mos til major molec response then q3-6 mos

94
Q

by what point should CML patient have major molec response = qPCR<0.1%

A

12 months

95
Q

what if pt has suboptimal response or tx failure?

A

-do mutation testing to guide alternative TKI to use

Haem/Onc (30 decks)

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