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Flashcards in ALL Deck (32)
1

Name the syndrome associated with Hypodiploid ALL

Li Fraumeni syndrome
50% germline mutation

2

Leukemia subtype proportions according to age

0-5 years: ALL:81% AML 14% CML 2% JMLL 3%
15-19 years: ALL:51%, AML:36%, CML:9%, JMLL:4%

3

Concordance rates in identical twins

100% if less 12 months
10-15% if more than 12 month

4

Name 5 syndromes associated with ALL

- Li Fraumeni syndrome
- Ataxia telangiectasia (T-ALL, NHL)
-NF1
- Bloom syndrome
- Down syndrome
- Kleinfelters

5

What is the most common cytogenetic abnormality in DS-ALL

CRLF2-R; P2RY8-CRLF2

6

Immunophenotype of Pre-B ALL

TdT+, CD10+, CD19+, CD22+, CD79a+

7

Immunophenotype of T-ALL

cCD3+, s CD3+, CD7+, CD2+, CD4+, CD8+

8

preB-ALL expresses CD13+, CD33+ molecular rearrangement is associated with?

- ETV6-RUNX1
- Ph+
- MLL ( less common)

9

Typical immunophenotype of Infant ALL

-CD10-

10

Burkitt's Leukemia Immunophenotype

Surface Ig

11

What is the characteristic translocation of Burkitt's Leukemia?

- t(2;8), t(8;14), t(8:22)
- c-Myc to heavy/light chain locus

12

Black, old teenager boy

T-cell ALL

13

Hypereosinophilia at diagnosis of ALL

t(5;14)(q31;q32) IL-3 gene under control of IgH promoter.
Can be a rare presentation of ALL

14

Name 8 x-rays findings associated with ALL

- Osteolysis
- Methaphyseal bands
- Osteopenia
- Osteosclerosis
- Permeative pattern
- Pathological fracture
- Periostial reaction
-Mixed lysis-sclerosis lesions

15

40% of children with ALL will complain of this symptom

Bone pain

16

ALL- Definition of CNS involvement

CNS1: NO blast in cytospin, regardless of the number of WBC.
CNS2:Presence of <5/uL WBC and cytospin positive for blast OR >=WBC but negative for Steinherts/Bleyer algorithm
CNS3: >+ 5/uL WBC and cytospin positive for blasts and/or clinical signs of CNS involvement.

17

Name 5 signs/symptoms of CNS involvement

- Cranial nerve palsies
- Headache
- Lethargy
- Seizures
- Papilledema

18

Name 3 subtypes of ALL with high incidence of CNS involvement

- Infant ALL (15-25%)
- Burkitt's Leukemia (15-25%)
- T- ALL (15%)

19

Name 5 negative prognostic factors at the time of relapse

- Short time to relapse <18 months from diagnosis
- Isolated bone marrow relapse
- Age at initial diagnosis (worse for teenagers that relapse)
- T-ALL relapse
-

20

Immunophenotype of Pro-B ALL

CD19+, CD10-, cIG-, sIg-

21

Re-arrangement associated to CD10 Negative leukemia

t(4;11)

22

Cytogenetic abnormalities in ALL

- ETV6-RUNX1 aka TEL-AML1 25% ( Most common)- Good outcome- t(12;21)
- TCF3-PBX1 aka E2A-PBX1 5% t(1;19) - neutral outcome
-MLL-rearranged 3% t(?;11) or t(11;?)- Poor outcomes
-BCR-ABL1 t(9;22) Poor outcome add TKI
- MYC- IgH, Igl, Igk t(8;14), t(2;8), t(8;22) Burkitt's

23

Define NCI risk groups:

- SR- Age: 1-9.99 WBC <50
- HR: Age <1>10 WBC >50

24

Prognostic factors:

- Age
- WBC
- Genotype
- Treatment response- Strongest prognostic factor

25

Outcomes of different risk groups

SR: EFS: 90%
HR: EFS: 75-80%

26

Hyperdiploid ALL

Excellent outcome
Trisomy 4, 10 and 17

27

Hypodiploid ALL

Poor outcome

28

Ph+ ALL Outcomes with TKI

7-yr EFS 75% ALLL0031

29

ALL incidence

20-80 cases/million ( 3 and 10 year old respectively)

30

Treatment of CNS leukemia

1800 cGy cranial irradiation and IT chemotherapy

31

Name 5 complications of IT chemotherapy

- Arachnoiditis
- Seizures
- Stroke
- Subacute encephalopathy
- Aseptic meningitis

32

Late complications of CNS-directed therapy

- Leukoencephalopathy
-Cortical atrophy
- Intellectual impairment
- Development of secondary brain tumors ( 10+ years after ttm- meningioma)
- Metabolic and endocrine changes