all Flashcards
childhood leukemia epidemiology- peak age, more common race and gender
age 1-4, W >B, boys more than girls
what race/ethnic group has inferior survival of ALL
black race and hispanic ethnicity
2 toxins that increase risk of ALL
mycotoxin and hydrocarbons (benzene)_
7 major genetic syndrome that I create risk of ALL
T21, NF1, bloom, falcon anemia (All less than AML), ataxia telangiectasia, Li fraumeni (aka TP53), and constitutional mismatch repair issues
4 genes for constitutional mismatch repait
MLH1 ,MSH2 .MSH6, PMS2
increased risk for ALL with T21
20-30 fold
common genetic change in T21 ALL but not alayws
`CRLF2
leukemia concordance in monozygotic twins: if index is <1 year old, if index is 1-6, and if index is more than 6
<1: nearly 100% (KMT2a), 1-6: 10-20%, lower latency, and then more than 6 is very unlikely
cytogenetic change associate with coagulopathy t diagnosis
17;19
define M1 marrow2
<5% blasts
define M2 marrow
5-25% blasts
what type of marrow is leukemia by definition
M3 (more than 5% blasts0
define CNS2
less than 5 CSF WBC and blasts are present
what is CNS3
blasts present, ,more Etna 5 wb per microliter
Steinherz/Bleyer algorithm
is CNS3 if CSF WBC/CSF RBC > 2X peripheral blood WBC/ blood RBC
when to use steinherz algoritm
more than 10 RBC in CNS
4 immunologic subtypes of B ALL
pro B, common B, pre B. mature B
what does pro B have on surface
CD79a, CD19, CD22 (at lesat 2)
What cell surface marker often predicts KMT2a R
negative for Z D10
what does common B express on Cell
CD10
what doe Pre B press
cytoplasmic immunoglobulin heavy chain (mu)
mature B has what
surface cell immunoglobulin
Which leukemia expresses HLA-DR
almost al pre B ALL, most T cell are NOT, some AMLs are
is TDT positive in b ALL
usually
MPO+ is what
MPAL
T cell ALL always expresses what and usually expresses what 2 other things
always cytoplasmic CD3 (cCD3+0, usually sCD3+ and TdT+, often also CD1a, 2 5 7
age group for SR leukemia
1-9
name 2 favorable genetic alterations in B ALL
hyperdiploid (N >50-53 and or favorable trisomies 4 and 10) AND ETV6 RUNX1 (t(12.21)
what is the one neutral genetic alternation in b all
t (1;19) (q23, p13.3) the TCF3 (E2A) PBX1 and this is more common in Black people
what is the pH+ chormosome
t(9;22) (q34; q11.2)
what is KMT2a
t(11q23; Variable)
what is the TCF3 rearrangement that is unfavorable
t(17;19)((q23, p13.3) TCF3 with HLF
is iAMP21 favorable or not
NO
what comprises 3 drug induction ALL
VCR< steroids, asparaginase
what is the 4 drug induction
VCR< steroids, asparaginase, daunorubicin
2 categories of Ph like subtypes
ABL like (15%) vs CRLF2 lik (half of them, this is the one with focmmon aissues with JAK)
what drug can you use for ABL ike fusion`
dasatanib or imatinib
what drug can you use for CRLF2 rearrangemnts
Ruxo
name a BiTE and how it works
Blina- connects CD3 to CD10
name a drug antibody conjugation and how it works
ino- targets CD22 an then gets calicheadmicin into it
what is ADE
cytarabine, daunt, etoposidew
how to risk stratify T all
CNS3 gu really post remission MRD and end of consolidation MRD is more important
what steroid is needed for T all
dexamethasone- slows relapse and improves OS
how to treat MPAL
all therapy unless you have a key AML genetic targetm
timing somnolence syndrome post CNS radiatino
5-7 weeks
how to time MTX with CNS XRT
give MTX first
imaging changes from cranial XRT
calcifications. you can get other issues
what site is better for relapse of ALL
isolated extra medullary is bette than bone marrowh
what is daratumumab
antibody to CD38
time to HSSCT if relapse B ALL
if <36 months from initial diagnosis or isolated extramedulary <18 months
what type of leukemia is more communion the following genetic syndromes: bloom NF, ataxia telangiectasia
bloom (AML >ALL), NF (AML >ALL), Ataxia telangiectasia (T/ALL or LLY)
loeffler syndrome
hypereosinophilia (could be rare presenting cause of leukemia) with CHF, pulmonary infiltrates, cardiomegaly
two types of AML with eosinophilia
either eosinophilic myeloid leukemia (M6 AML) or non-M6 with hypereosinophilia (characteristic rearrangement of chromosome 16)
Features of mature B all (aka bursts)
t(2;8), t(8;14), t(8;22)– all are c-myc translations to heavy or light chain locus
cd45 marks
hematopoietic
name 4 immature (myeloid and lymphoid) markers
HLA DR, TDT lymphoid) CD117, CD34
CD79a is
b lineage
cd99
t lineage
9 myeloid markers
CD13, 15, 33, 64/65, 11b (monocytic), 14 (monocytic), 163 (monocytic), MPO, NSE (monocytic)
what antigen receptors gene rearrangements exist in T all
T ALL- almost all TCR, only 10% have Ig rearrangements
CD10 lymphoblasts have what myeloid activity
TdT
which is less mature- early B or Pre B
early is less mature- no cytoplasmic Ig or surface. Pre B is more mature, has cIg but not sIg
how do you treat mature B cell ALL
like a advanced stage burkitts
what’s the ETV6-RUNX1 fusion
t(12;21)(p13;q22)
2 most common mutations in T ALL
activating NOTCH 1 and deletions in CDKN2A (9q21)
define hypodiploid
N <40-43
define hyperdiploid
N >50-53 and/or favorable trisomy (4 and 10) OR DNA index 1.16 or 53+ chromosomesw
where do you radiate and at what dose for CNS with leukemia
whole brain and posterior halves of globes of eyes. pox dose is 1200 cGy, treatment for CNS3 is 1800 cY in 10 fractions for maintenance
management testicular relapse ALL
chemo + 2400 cGy radiation
time for stroke like symptoms after IT therapy
7-11 ay
timelime osteonecrosis with ALL
most within 2 years of diagnosis
