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H/O RC > ALL > Flashcards

ALL Flashcards

1
Q

5 ALL etiology RF

A

ionizing radiation
EBV
prior chemotherapy
prior RT
genetic predisposition

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2
Q

ALL associated genetic syndromes

A

T21
LFS (hypodiploid)
A-T (T-ALL)
Bloom
FA
Family constitutional deficiencies ETV6 and PAX5

NF1, WAS, DBA, DKC, SDS

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3
Q

ALL familial RF

A

sibling with ALL, 2-4x

identical twin with ALL
- <1yo 100%
- >1yo 10%

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4
Q

ALL vs AML path/ICH differentiation

A

AML: MPO, auer rods (50% of AML), CD33, CD13, CD117

ALL: CD19, cytoplasmic CD79a, CD22, CD10, CD22, CD24, PAX5, Tdt, HLA-DR +

T-ALL: CD19 and sIg neg, , cCD3 (diagnostic), CD2/3/4/5/7/8 +

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5
Q

ALL prognostic factors

A

MRD +
age <1, 10+yo
initial WBC (>50)
cytogenetics

CNS +
testicular

F>M
T21

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6
Q

ALL fav/unfav cytogenetics

A

favourable
- ETV6-RUNX1 t(12;21) (20%)
- double trisomy 4/10
- hyperdiploid (21%): >2N, DNA index >1.16, >53 chromosomes

Unfavourable
- Ph+ t(9;22) (4%)
- Ph-like
- t17;19
- hypodiploid (<2N, DNA index <0.81, <44 chromosomes)
- iAMP21
- KMT2A (3% but 70%<1yo)

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7
Q

ALL CNS classification

A

CNS1: no blasts

CNS2:
- <5WBC, cytospin +
- >5WBC, cytospin +, negative w RBC correction
- traumatic LP

CNS3: 5+ blasts and/or clinical signs

Steinherz-Bleyes
+ if (CSF WBC/RBC) > 2x (serum WBC/RBC)

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8
Q

ALL differences in infants

A

increased KMT2Ar
higher WBC/hyperleukocytosis
more extramedullary disease
poorer prog

CD10 neg typically

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9
Q

ALL COG Risk Stratification

A

HR: <1/10+yo, WBC >50, MRD+ (>0.01%), unfav cytogenetics

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10
Q

ALL relapse prognostic factors

A

site (marrow worse than extramedullary)

timing
- very early <18mo
- early 18-36mo
- late >36 mo

Other:
- phenotype (T-ALL marrow worse)
- Primary dx px factors
- MRD

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11
Q

ALL CNS+ RF (upfront and relapse)

A

upfront: T-ALL, t(1;19), WBC>50, infant

at relapse: T-ALL, high WBC, initial CNS+, traumatic LP at dx, missed LPs

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12
Q

B-ALL relapse risk stratification and Tx (including HSCT indications)

A

LR: late (>18mo) IEM
- no HSCT

IR: late (>36mo) BM, early (<18mo) IEM

HR: early (<36mo) BM

HSCT for early (<36mo) marrow, early (<18mo) IEM, IR with EOI MRD +
(also for T-ALL relapse)

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13
Q

ALL CNS relapse RT dose

A

1800 cGy CRT including posterior halves of globes of eyes

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14
Q

ALL CNS-directed therapy

A

MTX (IT, HD, Capizzi)
Steroid (dex > pred)
asparaginase (functional)
CNS RT

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15
Q

ALL long term sequelae

A

SMN (2%) - cyclo, RT
peripheral neuropathy - VCR
white matter atrophy
mental helath
neuropsych - MTX, RT, IT
metabolic syndrome/obesity
HTN
relapse
short statue - steroid, TBI
osteopenia - steroid, TBI
cardiotoxicity (1-2%) - anthracyclines
infertility
cataracts - steroid, TBI
endocrinopathies

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16
Q

ALL - additional drugs for infant and T-ALL

A

infant: cytarabine

T: bortezomib, nelarabine, cytarabine

17
Q

ALL CRT indications

A

HR: CNS3
SR: T21 CNS3
Relapse

1800 cGy including posterior halves of globes

18
Q

ALL Tx adjustments in T21

A

leucovorin after IT
MTX dose reduction (ID)
CNS3 gets RT
Supportive care
- IVIG replacement
- in hospital for intensive phases

19
Q

CRS etiology, presentation, tx

A

blinatumomab, haplo transplant, CAR-T

fever, myalgia, NVD -> hypotension, resp/renal injury, coagulopathy

Tx: steroid, tocilizumab, supportive care

20
Q

ALL BMT indications

A

induction failure
EOC MRD+
t(17;19)
HR relapse
T relapse

21
Q

3 Ph-like mutation classes

A

ABL-like: dasatinib
JAK-STAT alterations (CRLF2)
RAS activating

22
Q

ALL diploid groups and associations

A

near haploid: 24-31 chromosomes
- younger, RAS mutations, worse px

low hypodiploid: 23-29 chromosomes
- rare, TP53 mutations

high hypodiploid: 40-43 chromosomes

Diploid

Low Hyperdiploid: 47-50 chromosomes
High hyperdiploid: 51-67 chromosomes

23
Q

ALL PAX5 abnormalities

A

PAX5alt (7%)
PAX5 P80R: signaling pathway mutations

Both EFS/OS mid 70s

24
Q

TPMT testing modifications

A

heterozygous: can start at full dose
homozygous: start at 10% dosing

25
Q

HR risk stratification

A

HR-fav: <10yo, CNS1, favourable cyto, MRD neg

HR

VHR: EOI/EOC MRD +

26
Q

Ph+ ALL risk stratification and tx

A

IB is induction 2 (similar to B-ALL SOC consolidation)

SR: end IB MRD neg (<0.05%)

HR (20%): end IB MRD + (>0.05%); HSCT in CR1

27
Q

Infant ALL risk stratification and prognosis

A

HR: KMT2A-r and <3mo; EFS/OS 21/30%
IR: KMT2A-r; EFS/OS 45/58%
LR: wt-KMT2A; EFS/OS 74/87%

28
Q

T-ALL cytogenetics

A

none are prognostic

NOTCH1 activating mutations (60%)
loss of CDKN2A (del9p) (70%)
TAL1 (40%)

29
Q

T-ALL risk stratification

A

SR: EOI MRD neg, CNS1, no test, no CS pretreatment

IR: EOI MRD + (>0.01%) but EOC MRD neg (<0.1%)

VHR: EOC MRD + (>0.1%) or induction failure

30
Q

T-ALL CRT indications

A

IR (EOI MRD + but EOC neg) CNS3
VHR any CNS status - 1200 cGy

31
Q

FORUM trial question and results

A

4-21yo
12Gy TBI + etop vs Flu/Thio/Bu or Treo

TBI superior - OS 91 vs 86%, RR 12 vs 33%

H/O RC (31 decks)

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