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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
How long is maintenance therapy?
2 years for girls from start of IM1 3 years for boys from the start of IM1
26
For HR and VHR ALL treatment how is induction different from standard?
Daunomycin is added | Prednisone for those >10yrs, dexamethasone for those that are younger but only 14 days, however use 10mg/m2
27
For HR and VHR ALL treatment how is consolidation different from standard?
Lasts 9 weeks Cyclo/AraC added Peg-asp added (SR has VCR, 6-MP, 3xIT)
28
For HR and VHR ALL treatment how is in | IM1 different from standard?
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
For HR and VHR ALL treatment how is DI different from standard?
2 doses of Peg-asparaginase
30
What poor prognostic factors are associated with infant ALL?
``` High initial WBC Massive organomegaly Thrombocytopenia Cns leukaemia Failure to achieve complete remission by day 14 ```
31
Where is the MLLgene located?
Band 11q23
32
What adverse independent prognostic features are associated with infant ALL?
Age less than 3 mth High wbc Slow response to induction Presence of translocation at 11q23
33
ALL in trisomy 21 is associated with which cytogenetic subtypes?
Jak2 mutation Fusion between P2RY8 and CRLF2 Absence of hyperdiploid, ETV6-RUNX1
34
ALL in trisomy 21 is rarely or almost never associated with this subtype?
T-cell ALL
35
Infant ALL negative risk factors
WBC > 300 Age < 3 months MLL
36
ALL favourable prognostic factors
``` NCI - Age 1-10, WBC < 50 Immunophenotype - B>T (less so now) Hyperdiploid (DNA 1.16) (12;21) ETV6-RUNX1 MRD negative ```
37
ALL negative prognostic factors
``` 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 & DIC iAMP 21 IKZ mutations MRD positive ```
38
Genetic conditions associated with ALL
``` Congenital agammaglobulinemia Poland syndrome Shwachmann Diamond syndrome Ataxia telangiectasia Li-Fraumeni Neurofibromatosis Diamond-Blackfan Kostmann disease Bloom syndrome ```
39
Options for CNS directed therapy
Intrathecal chemotherapy Irradiation Systemic chemotherapy - dexamethasone, MTX Systemic depletion of asparagine - L-asparaginase does not penetrate CNS
40
Indications for HSCT in CR1 for ALL
Hypodiploid ALL (<44 chromosomes) End consolidation MRD > 0.01 Induction failure MLL + Infant ALL (controversial)
41
What is the risk of developing ALL in identical twins less than 12 months old?
100% | Believe that there is transplacental transfer of cells with a chromosomal translocation leading to ALL
42
Patients with Ataxia Telangiectasia are at risk for which type of ALL?
T-ALL | and also T-NHL
43
Are patients with Neurofibromatosis more likely to develop AML or ALL?
AML>>>ALL
44
CD45 characterizes which type of cell?
Hematopoietic cells
45
What is the most common immunophenotype for pre-B ALL?
``` CD10+ CD19+ CD22+ CD79a+ Can express myeloid markers CD13+ or CD33+ ```
46
Is it common for pre-B ALL to be HLA-DR+
Yes almost all are positive | compared to to T-ALL where most are HLA-DR negative
47
What is a common immunophenotype for T-ALL
cytoplasmic CD3+, often surface CD3+, CD7+, Tdt+ Can express CD2,4,8 CD10 expression is variable
48
Is pre-B ALL surface Ig positive or negative
Negative! | Being positive is a mature B cell marker, ie Burkitts leukemia is surface Ig positive
49
Which translocation is associated with ALL and hypereosinophilia?
t(5;14) | eosinophilia is reactive and not part of the leukemic clone
50
List a differential diagnosis for pancytopenia in childhood
``` Leukemia Metastatic solid tumour Aplastic anemia HLH MDS/myelofibrosis Infectious or post-infectious Lupus hypersplenism B12 or folate deficiency ```
51
What prognostic factors are important for ALL relapse?
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
When can isolated CNS relapse patient be treated with systemic therapy and radiation alone?
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
What is blinatumomab
A bi-specific T-cell engager monoclonal antibody against CD19
54
What pre-disposition syndrome is associated with low hypodiploid ALL?
p53/Li Fraumeni
55
What surface and cytoplasmic Ig are expressed in pro-B ALL?
``` CD19+ CD10- cIg- sIg- Often M-R ```
56
What surface and cytoplasmic Ig are expressed in Pre-pre-B ALL?
CD19+ CD10+ cIg- sIg-
57
What surface and cytoplasmic Ig are expressed in pre-B ALL?
CD19+ CD10- cIg+ sIg-
58
What surface and cytoplasmic Ig are expressed in nature B (Burkett) ALL?
CD19+ CD10+ or - cIg + or - sIg+
59
What is lack of CD10 expression commonly lined with?
MLL rearrangement
60
What are 4 findings on X-ray that you might expect to see on a patient with ALL?
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
What is the Steinherz-Breyer equation used for? And what is it?
Used to determine significance of a bloody tap. | CSF WBC/CSF RBC > 2x Blood WBC/Blood RBC
62
What is the genetic polymorphism that catalyzes the inactivation of mercaptopurine?
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
AALL0331 - LR trial: main outcomes?
Intensified Consolidation did not improve SR-Avg pts
64
AALL0232 - HR trial: main outcomes?
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
AALL0434 - T-ALL trial : main outcome?
Capizzi-Style Methotrexate with Pegasparagase (C-MTX) Is Superior to High-Dose Methotrexate (HDMTX) in T-Lineage Acute Lymphoblastic Leukemia (T-ALL):
66
Interfant 99 - main outcomes?
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
List 3 modifications to therapy or management in Down syndrome ALL
- 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
What additional therapy do HR B-ALL patients receive compared to standard risk?
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
ETP immunophenotype
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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