leukaemia / lymphoma Flashcards
(42 cards)
most common childhood cancer
ALL
most common type of ALL
B-ALL (85%)
T-ALL (15%)
peak age of ALL presentation
2-5y
4 syndromes a/w ALL
a. Down syndrome
b. NF1
c. Bloom syndrome
d. Ataxia telangiectasia
biggest prognostic factor for ALL
response to induction!!
ALL: favourable vs not favourable genetics
favourable:
1. Hyperdiploidy (>50)
2. Trisomies 4, 10
3. ETV-RUNX protein (t12;21)
not favourable:
1. Hypodiploidy (<44)
2. Philadelphia t(9;22)
achieving remission after end of induction measured by?
MRD - must be <0.01% by D29
ALL risk factors
i. Age > 10y or <1y
ii. WCC > 50 or CSF/testicular involvement at diagnosis
iii. Cytogenetic/molecular
iv. Response to induction – biggest prognostic factor
for FRACP, DIC + leukaemia = what kind?
APML
very specific FRACP features for AML
- Subcutaneous nodules – ‘blueberry muffin’ lesions
- Infiltration of gingiva
- DIC (APML**)
- Discrete masses – chloromas, granulocytic sarcomas
bone marrow findings of ALL vs AML
ALL 85% blasts, high nucleus:cytoplasm ratio
AML 20% blasts, with auer rods
describe the mutation in APML, and therefore its treatment
t15;17 = PML-RARA: responsive to all-trans-retinoic acid (ATRA, tretinoin)
survival rate of ALL vs AML
ALL: up to 99% with favourable genetics, say 90%
AML: 60-70%
down syndrome: AML or ALL?
ALL: 20x more common EXCEPT in first 3 years of life
- worse cytogenetics, worse prognosis
- T21 more sensitive to MTX
AML: better survival in T21!
why are FBEs important in T21 neonates?
10% get transient leukaemia/MPD (high leuks, blasts, low plt/Hb)
and 20-30% will develop leukaemia by 3y
bcr-abl: what’s the mutation and Rx
t(9;22) > imatinib TK inhibitor
JMML syndromic risk factors
- noonan’s
- NF1
most JMML have mutations in what pathway?
RAS/MAP kinase pathway
key features of JMML
- 1/3rd present with an URTI with hepatosplenomegaly and a rash
- *monocytosis *
most cases of infant leukaemia (<1y) from what mutation?
> 80% MLL gene (11q23 band translocation; majority t(14;11)) - and this is a shit one
HLH: most common age group
birth to 18m
pathogenesis of HLH
trigger > abnormal immune activation: macrophages eat up RBC and make cytokine storm, but NK and CD8 T cells can’t shut the macrophages down
most familial HLH is caused by what kind of problem?
defect in perforin, so NK/CD8 can’t do their job against the macrophages
aetiology of HLH
- genetic mutation
- infection - EBV**
- malignancy - esp lymphoid
- macrophage activation syndrome e.g. JIA/other rheum
