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ALL Flashcards

1
Q

Name the syndrome associated with Hypodiploid ALL

A

Li Fraumeni syndrome

50% germline mutation

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2
Q

Leukemia subtype proportions according to age

A

0-5 years: ALL:81% AML 14% CML 2% JMLL 3%

15-19 years: ALL:51%, AML:36%, CML:9%, JMLL:4%

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3
Q

Concordance rates in identical twins

A

100% if less 12 months

10-15% if more than 12 month

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4
Q

Name 5 syndromes associated with ALL

A
  • Li Fraumeni syndrome
  • Ataxia telangiectasia (T-ALL, NHL)
  • NF1
  • Bloom syndrome
  • Down syndrome
  • Kleinfelters
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5
Q

What is the most common cytogenetic abnormality in DS-ALL

A

CRLF2-R; P2RY8-CRLF2

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6
Q

Immunophenotype of Pre-B ALL

A

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

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7
Q

Immunophenotype of T-ALL

A

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

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8
Q

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

A
  • ETV6-RUNX1
  • Ph+
  • MLL ( less common)
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9
Q

Typical immunophenotype of Infant ALL

A

-CD10-

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10
Q

Burkitt’s Leukemia Immunophenotype

A

Surface Ig

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11
Q

What is the characteristic translocation of Burkitt’s Leukemia?

A
  • t(2;8), t(8;14), t(8:22)

- c-Myc to heavy/light chain locus

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12
Q

Black, old teenager boy

A

T-cell ALL

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13
Q

Hypereosinophilia at diagnosis of ALL

A

t(5;14)(q31;q32) IL-3 gene under control of IgH promoter.

Can be a rare presentation of ALL

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14
Q

Name 8 x-rays findings associated with ALL

A
  • Osteolysis
  • Methaphyseal bands
  • Osteopenia
  • Osteosclerosis
  • Permeative pattern
  • Pathological fracture
  • Periostial reaction
  • Mixed lysis-sclerosis lesions
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15
Q

40% of children with ALL will complain of this symptom

A

Bone pain

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16
Q

ALL- Definition of CNS involvement

A

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
Q

Name 5 signs/symptoms of CNS involvement

A
  • Cranial nerve palsies
  • Headache
  • Lethargy
  • Seizures
  • Papilledema
18
Q

Name 3 subtypes of ALL with high incidence of CNS involvement

A
  • Infant ALL (15-25%)
  • Burkitt’s Leukemia (15-25%)
  • T- ALL (15%)
19
Q

Name 5 negative prognostic factors at the time of relapse

A
  • 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
Q

Immunophenotype of Pro-B ALL

A

CD19+, CD10-, cIG-, sIg-

21
Q

Re-arrangement associated to CD10 Negative leukemia

A

t(4;11)

22
Q

Cytogenetic abnormalities in ALL

A
  • 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
Q

Define NCI risk groups:

A
  • SR- Age: 1-9.99 WBC <50

- HR: Age <1>10 WBC >50

24
Q

Prognostic factors:

A
  • Age
  • WBC
  • Genotype
  • Treatment response- Strongest prognostic factor
25
Q

Outcomes of different risk groups

A

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

26
Q

Hyperdiploid ALL

A

Excellent outcome

Trisomy 4, 10 and 17

27
Q

Hypodiploid ALL

A

Poor outcome

28
Q

Ph+ ALL Outcomes with TKI

A

7-yr EFS 75% ALLL0031

29
Q

ALL incidence

A

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

30
Q

Treatment of CNS leukemia

A

1800 cGy cranial irradiation and IT chemotherapy

31
Q

Name 5 complications of IT chemotherapy

A
  • Arachnoiditis
  • Seizures
  • Stroke
  • Subacute encephalopathy
  • Aseptic meningitis
32
Q

Late complications of CNS-directed therapy

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

ALL (3 decks)

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