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SADL Oncology > ALL > Flashcards

ALL Flashcards

1
Q

What are important factors in the pathogenesis of Leukemia?

A

Ionizing radiation
Chemicals
Drugs-ie alkylating agents
Genetics
-twins-in 1st 5yrs of life the risk of the second twin is 20%
-siblings the risk is 4x greater than general population
-chromosome abnormalities

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2
Q

What is the risk of developing leukaemia in a Down syndrome patient before the age of 10yrs?

A

1 in 95

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3
Q

What is the definition of CNS1 for ALL?

A

<5 WBC/mm3

No blasts on spin

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4
Q

What is the definition of CNS2?

A

<5 WBC/mm3 and blasts on cytospin

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5
Q

What is the definition of CNS3?

A

> 5 WBC/mm3 and blasts on the slide

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6
Q

For a traumatic LP Tap what equation should be used to determine if cns disease is present?

A

CNS disease is present if: CSF WBC/CSF RBC >2x blood WBC/blood rbc

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7
Q

ETV6-RUNX1 fusion gene involves which 2 chromosomes

A

t(12;21)

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8
Q

BCR-ABL fusion gene is associated with what features at diagnosis?

A

Older age
Higher WBC
Cns involvement

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9
Q

What translocations involving the MYC genes are seen in mature B-cell ALL

A

8;14 is most common

Then 2;8 and 8;22

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10
Q

What does the NOTCH1 gene code for?

A

Encodes a transmembrane receptor that regulates normal t-cell development

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11
Q

What are the 2 most NB predictors of outcome?

A

Age and WBC

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12
Q

List the prognostic factors for ALL

A 
Age
WBC
Immunophenotype
Cytogenetics
DNA index
Response to induction
CNS status
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13
Q

What is an M1 marrow

A
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14
Q

What is an M2 marrow?

A

5-25% blasts

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15
Q

What is an M3 marrow?

A

> 25% blasts in marrow

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16
Q

What is the lowest level of MRD detection by morphology

A

5 leukaemia cells per 100 normal bone marrow cells

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17
Q

What is the level of detection of MRD by multicolour flow cytometry?

A

1 per 10,000

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18
Q

What is the level of detection of MRD by PCR?

A

1 per 1,000,000

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19
Q

What drugs are used in standard risk ALL therapy

Induction?

A 
Dexamethasone
Vincristine 
Asparaginase
IT cytarabine
IT MTX
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20
Q

What drugs are used in standard risk ALL therapy

Consolidation?

A

6-mp
Vincristine on day 1
IT MTX

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21
Q

What drugs are used in standard risk ALL therapy

Interim maintenance

A

Capizzi MTX

Vincristine

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22
Q

For capizzi MTX what is the dose escalation and when do you not escalate

A

Escalate by 50mg/m2/dose on days 11,21,31,41

Stop escalation and resume at 80% of last dose if there is a delay because of mucositis or myelosuppression

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23
Q

What drugs are used in standard risk ALL therapy

Delayed intensification?

A 
Dexamethasone
Vincristine
Doxo day 1,8,15 25mg/m2
Cyclophosphamide day 29 1000mg/m2
6-TG
IV cytarabine 2, 4 day courses
IT MTX
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24
Q

What drugs are used in standard risk ALL therapy

Maintenance ?

A 
Dexamethasone 5 day courses at beginning of each month 
Vincristine 
6-MP
Oral MTX, omit on IT days
IT MTX
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25
Q

How long is maintenance therapy?

A

2 years for girls from start of IM1

3 years for boys from the start of IM1

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26
Q

For HR and VHR ALL treatment how is induction different from standard?

A

Daunomycin is added

Prednisone for those >10yrs, dexamethasone for those that are younger but only 14 days, however use 10mg/m2

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27
Q

For HR and VHR ALL treatment how is consolidation different from standard?

A

Lasts 9 weeks
Cyclo/AraC added
Peg-asp added

(SR has VCR, 6-MP, 3xIT)

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28
Q

For HR and VHR ALL treatment how is in

IM1 different from standard?

A

Lasts 63 days
Get high dose MTX 5g/m2 with leucovorin rescue at hour 42 after the start of HD MTX infusion
Oral 6-MP

29
Q

For HR and VHR ALL treatment how is DI different from standard?

A

2 doses of Peg-asparaginase

30
Q

What poor prognostic factors are associated with infant ALL?

A 
High initial WBC
Massive organomegaly
Thrombocytopenia 
Cns leukaemia 
Failure to achieve complete remission by day 14
31
Q

Where is the MLLgene located?

A

Band 11q23

32
Q

What adverse independent prognostic features are associated with infant ALL?

A

Age less than 3 mth
High wbc
Slow response to induction
Presence of translocation at 11q23

33
Q

ALL in trisomy 21 is associated with which cytogenetic subtypes?

A

Jak2 mutation
Fusion between P2RY8 and CRLF2

Absence of hyperdiploid, ETV6-RUNX1

34
Q

ALL in trisomy 21 is rarely or almost never associated with this subtype?

A

T-cell ALL

35
Q

Infant ALL negative risk factors

A

WBC > 300
Age < 3 months
MLL

36
Q

ALL favourable prognostic factors

A 
NCI - Age 1-10, WBC < 50 
Immunophenotype - B>T (less so now)
Hyperdiploid (DNA 1.16) 
(12;21) ETV6-RUNX1
MRD negative
37
Q

ALL negative prognostic factors

A 
NCI - Age < 1 or >10, WBC > 50
CNS positive
Trisomy 21
Hypodiploid (<44 chrom)
t(9;22) BCR-ABL1
Ph-like ALL
t(v;11q23) MLL (KMT2A) - (4;11) is most common
t(1;19) and t(17;19) - associated with hypercalcemia &amp; DIC
iAMP 21
IKZ mutations
MRD positive
38
Q

Genetic conditions associated with ALL

A 
Congenital agammaglobulinemia
Poland syndrome
Shwachmann Diamond syndrome
Ataxia telangiectasia
Li-Fraumeni
Neurofibromatosis
Diamond-Blackfan
Kostmann disease
Bloom syndrome
39
Q

Options for CNS directed therapy

A

Intrathecal chemotherapy
Irradiation
Systemic chemotherapy - dexamethasone, MTX
Systemic depletion of asparagine - L-asparaginase does not penetrate CNS

40
Q

Indications for HSCT in CR1 for ALL

A

Hypodiploid ALL (<44 chromosomes)
End consolidation MRD > 0.01
Induction failure
MLL + Infant ALL (controversial)

41
Q

What is the risk of developing ALL in identical twins less than 12 months old?

A

100%

Believe that there is transplacental transfer of cells with a chromosomal translocation leading to ALL

42
Q

Patients with Ataxia Telangiectasia are at risk for which type of ALL?

A

T-ALL

and also T-NHL

43
Q

Are patients with Neurofibromatosis more likely to develop AML or ALL?

A

AML»>ALL

44
Q

CD45 characterizes which type of cell?

A

Hematopoietic cells

45
Q

What is the most common immunophenotype for pre-B ALL?

A 
CD10+
CD19+
CD22+
CD79a+
Can express myeloid markers CD13+ or CD33+
46
Q

Is it common for pre-B ALL to be HLA-DR+

A

Yes almost all are positive

compared to to T-ALL where most are HLA-DR negative

47
Q

What is a common immunophenotype for T-ALL

A

cytoplasmic CD3+, often surface CD3+, CD7+, Tdt+
Can express CD2,4,8
CD10 expression is variable

48
Q

Is pre-B ALL surface Ig positive or negative

A

Negative!

Being positive is a mature B cell marker, ie Burkitts leukemia is surface Ig positive

49
Q

Which translocation is associated with ALL and hypereosinophilia?

A

t(5;14)

eosinophilia is reactive and not part of the leukemic clone

50
Q

List a differential diagnosis for pancytopenia in childhood

A 
Leukemia
Metastatic solid tumour
Aplastic anemia
HLH
MDS/myelofibrosis
Infectious or post-infectious
Lupus
hypersplenism
B12 or folate deficiency
51
Q

What prognostic factors are important for ALL relapse?

A

site- marrow worse than extramedullary
timing-early worse than late
age at initial dx-older worse than younger
immunophenotype- T-ALL tough to cure

52
Q

When can isolated CNS relapse patient be treated with systemic therapy and radiation alone?

A

If the relapse is longer than 18 months from the first remission. If relapse is earlier than the first remission they should be put into remission and then undergo HSCT

53
Q

What is blinatumomab

A

A bi-specific T-cell engager monoclonal antibody against CD19

54
Q

What pre-disposition syndrome is associated with low hypodiploid ALL?

A

p53/Li Fraumeni

55
Q

What surface and cytoplasmic Ig are expressed in pro-B ALL?

A 
CD19+
CD10-
cIg-
sIg-
Often M-R
56
Q

What surface and cytoplasmic Ig are expressed in Pre-pre-B ALL?

A

CD19+
CD10+
cIg-
sIg-

57
Q

What surface and cytoplasmic Ig are expressed in pre-B ALL?

A

CD19+
CD10-
cIg+
sIg-

58
Q

What surface and cytoplasmic Ig are expressed in nature B (Burkett) ALL?

A

CD19+
CD10+ or -
cIg + or -
sIg+

59
Q

What is lack of CD10 expression commonly lined with?

A

MLL rearrangement

60
Q

What are 4 findings on X-ray that you might expect to see on a patient with ALL?

A

Osteolytic lesions involving medullary cavity and cortex.
Transverse metaphysical radiolucent bands.
Transverse metaphysical lines of increased density (growth arrest lines).
Subperiosteal née bone formation.

61
Q

What is the Steinherz-Breyer equation used for? And what is it?

A

Used to determine significance of a bloody tap.

CSF WBC/CSF RBC > 2x Blood WBC/Blood RBC

62
Q

What is the genetic polymorphism that catalyzes the inactivation of mercaptopurine?

A

Gene polymorphism for thiopurine methyltransferase.
10% of the population carries at least one variant allele. Results in high levels of the active 6MP metabolite, and increases side effects. Requires decreased doses.

63
Q

AALL0331 - LR trial: main outcomes?

A

Intensified Consolidation did not improve SR-Avg pts

64
Q

AALL0232 - HR trial: main outcomes?

A
  1. Interim analysis showed HD MTX better than capizzi. 5yr EFS 82%vs 75%.
  2. Pts <10yrs had better outcome with dex.
  3. Pts >10yrs dex showed no benefit, associated with more osteonecrosis, should receive prednisone
65
Q

AALL0434 - T-ALL trial : main outcome?

A

Capizzi-Style Methotrexate with Pegasparagase (C-MTX) Is Superior to High-Dose Methotrexate (HDMTX) in T-Lineage Acute Lymphoblastic Leukemia (T-ALL):

66
Q

Interfant 99 - main outcomes?

A
  1. Efficacy of a hybrid protocol demonstrated MLL rearrangement, age <6 months at diagnosis and poor day 8 prednisone response identified as independent adverse prognostic factors
  2. No benefit from adding a late intensification course
  3. Prognostic impact of MRD following induction and consolidation identified
  4. Risk of relapse significantly higher for congenital ALL
  5. HSCT beneficial for MLL-rearranged infants aged <6 months and poor day 8 prednisone response or WBC ≥300 g/l at diagnosis
67
Q

List 3 modifications to therapy or management in Down syndrome ALL

A
  • limit dose of HD mtx
  • Leucovorin with IT chemo
  • prophylactic IVig replacement
  • remain hospitalized until count recovery
  • prophylactic anti-fungal therapy
  • conservative F&N management
68
Q

What additional therapy do HR B-ALL patients receive compared to standard risk?

A

1) Dauno in induction
2) Intensified consolidation
3) IM#1 has HD-MTX
4) DI part 2 has additional VCR/Peg
5) IM#2 for VHR with 2 more Peg
6) Extra IT therapy in maintenance (even for CNS1)

69
Q

ETP immunophenotype

A

cCD3 +,
Positive for immature markers: TdT, CD117, or HLA-DR
CD1a, CD5, CD 8 negative
Positive for one or more myeloid marker

SADL Oncology (17 decks)

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