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Flashcards in ALL Deck (69)
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1

What are important factors in the pathogenesis of Leukemia?

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

2

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

1 in 95

3

What is the definition of CNS1 for ALL?

<5 WBC/mm3
No blasts on spin

4

What is the definition of CNS2?

<5 WBC/mm3 and blasts on cytospin

5

What is the definition of CNS3?

>5 WBC/mm3 and blasts on the slide

6

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

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

7

ETV6-RUNX1 fusion gene involves which 2 chromosomes

t(12;21)

8

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

Older age
Higher WBC
Cns involvement

9

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

8;14 is most common
Then 2;8 and 8;22

10

What does the NOTCH1 gene code for?

Encodes a transmembrane receptor that regulates normal t-cell development

11

What are the 2 most NB predictors of outcome?

Age and WBC

12

List the prognostic factors for ALL

Age
WBC
Immunophenotype
Cytogenetics
DNA index
Response to induction
CNS status

13

What is an M1 marrow

14

What is an M2 marrow?

5-25% blasts

15

What is an M3 marrow?

>25% blasts in marrow

16

What is the lowest level of MRD detection by morphology

5 leukaemia cells per 100 normal bone marrow cells

17

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

1 per 10,000

18

What is the level of detection of MRD by PCR?

1 per 1,000,000

19

What drugs are used in standard risk ALL therapy
Induction?

Dexamethasone
Vincristine
Asparaginase
IT cytarabine
IT MTX

20

What drugs are used in standard risk ALL therapy
Consolidation?

6-mp
Vincristine on day 1
IT MTX

21

What drugs are used in standard risk ALL therapy
Interim maintenance

Capizzi MTX
Vincristine

22

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

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

23

What drugs are used in standard risk ALL therapy
Delayed intensification?

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

24

What drugs are used in standard risk ALL therapy
Maintenance ?

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

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