ALL, ASPHO Flashcards
Name 2 ALL abnormalities that can arise in utero
- KMT2A=ALL
- Hyperdiploid ALL
What percent of baby with ETV/RUNX1 detectable on cord blood will develop ALL?
1%
For kids under 15, what % of leukemias are ALL? AML?
ALL=80%
AML=15%
For kids 15-19, what % of leukemias are ALL? AML?
ALL= 56% AML= 31%
what’s the peak age for ALL dx? AML?
ALL= 2-4; AML= no peak incidence
Childhood leuk represents what % of all new childhood cancer cases?
26.1%
Give 3 factors that have led to improved ALL survival
- CNS ppx started in 1970s
- Intensified therapies for identified risk groups with higher rates of relapse
- Improved supportive care
Give 6 ALL predispo syndromes
DNA CBNNRKL DNA NotBadLuck Down Syndrome NF1 Ataxia Telangiectasia (T-ALL) Noonan Syndrome Bloom Syndrome Li Fraumeni Syndrome
Also:
Constitutinal Robertsonia Translocation, Nijmegen Breakage syndrome, Constitutional Mismatch repair deficiencies, Klinefelter sydnrome
Risk increase for leukemia if T21?
20x
Higher risk of leukemia in T21 pertains to what age?
First 3 decades of life
Although the relative risk of AML is ___ than ____, ____ is more common than ___ except in the ___ yr of life
- higher
- ALL
- DS-ALL
- DS-AML
- first
what percent of children with B-precursor ALL have DS?
3%
Which common sentinel genetic alterations in ALL are less common if DS-ALL?
KMT2A translocations
ETV-RUNX1
BCR/ABL rearrangement
Trisomy 4+10
which sentinel genetic alteration in ALL is more common if DS-ALL?
CRLF2 overexpression+ accompanying JAK and IL7R mutations
which genetic mutation has better outcome in DS-ALL than non DS ALL?
CRLFr
in general who does better non-DS or DS ALL?
non-DS…unless you discount common genetic mutations, then they are the same
there’s more mortality due to what in DS-ALL?
toxic related death
give 5 germline mutations predisposing towards ALL
PAX5 G183S mutations TP53 (hypodiploid) ETV6 (hyper) FANCA (hypo) MLL3 (hypo)
what percent of childhood leukemia is associated with deleterious germline mutations?
~5%
Discuss concordance rates for ALL in identical twins by age…why? usually due to what genetic alteration in infants?
<12 mos: close to 100%
after 12 mos: 10-15%….concordance in infant twins due to early chrom translocations and transplacental trasnfer or leuk/preleuk cells to other twin…KMT2A
Give symptoms/signs of ALL
- Bone pain/refusal to walk
- Decreased energy, pallor, loss of appetite
- Adenoapthy/HSM
- mediastinal mass, SOB, unable to lie flat
- headache, neck pain, sz, CN Palsy if CNS invovled
- painless enlarged testes
TLS associated with what type of ALL
t-cell
risks of hyperleuk more in ALL or AML?
AML
What can genotype of ALL help determine? (3)
prognosis, tx stratification, and soemtimes use of targeted therapies (eg TKI for Ph+ ALL)