ALL facts Flashcards
CNS involvement in ALL diagnosis
5-10%
CNS relapse rates in ALL
15%
25% isolated
75% with full blown disease
Risk factors for CNS involvement in ALL diagnosis
elevated WBC (>30K B-ALL, >100K T-ALL)
LDH
High risk cytogenetics
T-ALL
Timing of 1st LP in ALL
debate between at diagnosis or after eradication of peripheral blasts
PLT threshold for LP
50-75K
CNS penetrating agents in ALL
MTX, cytarabine, dexamethasone, PEG-asp, 6-MP, and dasatinib
CNS presence of disease grading
CNS1- no blasts in CSF
CNS2 < 5 blasts in CSF
CNS3 >5 blasts in CSF
Tx of ALL with CNS disease at presentation
1/w or 2/w IT (MTX/Cytosar)
until clearance of blasts from CSF
TKI choice for Ph+ B-ALL with CNS involvement
Dasatinib has the most robust data
but ponatinib also crosses BBB
Dose of intra Ommaya chemo
50% than IT
Transplant decision in ALL with CNS involvement
Allo HCT at CR1
Ph+ ALL %
20-25%
increases to 50% after age 50
OS and PFS after D-ALBA in Ph+ ALL
90-95% in 18 months
70-80% after 3 years
IKZF1plus
IKZF1+
PAX5 or CDKN2A/2B
Agents effective in RR Ph+ ALL
Inotuzumab- CD22 conjugated ab-toxin
Blinatumomab- CD19 BiTE
Tisa cell, Brexu cell
ALL classification
B-ALL- with recurrent genetic abnormalities (Hypodiploid (usually p53) BCR:ABL1, KM2TA, ph-like, ETV6:RUNX1)
B- ALL/LBL-NOS
T-ALL/LBL (ETP)
B-ALL immunophenotype
Defining: CD19 + CD22/CD79a/CD10
PAX5, TdT positive
T-ALL immunophenotype
TdT, CD3,CD7, CD1a, CD4/CD8
ETP T-ALL immunophenotype
CD3+, CD5 weak, CD7+-
CD1a, CD4, CD8 neg
Myeloid/SC markers: HLA-DR, CD34, CD33, CD13, CD117 positive
Ph+ B-ALL incidence by age
increases with age