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CPHON Slides > 3 Childhood Leukemias > Flashcards

3 Childhood Leukemias Flashcards

1
Q

The myeloid and lymphoid cell lines are derived from the

A

pluripotent stem cells, that can differentiate into nearly all cells

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2
Q

Examples of chromosomal breakage syndromes that could cause children to be at increased risk of developing leukemia

A

Fanconi anemia, bloom syndrome, ataxia telangectasia, diamond blackfan, noonan syndrome, kostmann syndrome, klinefelters, dyskeratosis congenita, familial platelet disorder

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3
Q

ALL vs AML incidence

A

ALL 75% leukemia, and about 1/4 of childhood cancer. AML is about 15% childhood leukemia and about 5% of all childhood cancers

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4
Q

5 year relative survival rate of ALL? AML?

A

ALL - 89%, AML - 60%

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5
Q

‘extramedullary involvement’ means

A

blasts spilled outside of the marrow

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6
Q

What is a chloroma

A

leukemic cell infiltration sometimes found in soft tissues, bones etc. More commonly invading the skin in AML. They are slightly green when excised or exposed to air. Potential to cause cord compression or visual loss depending on placement.

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7
Q

What percent of children with leukemia will present with a normal CBC?

A

10%

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8
Q

Elevated potassium, phosphorus, and uric acid may be?
Low calcium or high creat?
Elevated LDH?

A

Could all be signs of tumor lysis syndrome

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9
Q

Signs of DIC? (lab values)

A

Elevated PT/PTT, low fibrinogen, elev D Dimer

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10
Q

When is it ideal to get HLA typing?

A

(only for high risk patients who might need transplant) prior to Chemo since it usually involves WBCs

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11
Q

All children with suspected leukemia have a 5-10% chance of presenting with ___ and should get a rule out ____

A

mediastinal mass, CXR. Could cause obstruction when doing initial procedures

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12
Q

4 major types of tests done on bone marrow at diagnosis

A

Morphology, Cytochemistry, Immunophenotyping, and cytogenetics

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13
Q

What is morphology?

A

The form and structure of the cells. Leukemic cells are ‘hypercellular/packed’ and percentage of blasts is determined,
Erythroid- RBC, Granulocytic - WBC, Megakaryocytic - PLT

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14
Q

What is cytochemistry?

A

helps determine ALL vs AML among other things, differentiates AML subtypes

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15
Q

What does immunophenotyping (aka flow cytometry) identify?

A

Antigens on blasts, also help differentiate AML and ALL as well as subtypes

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16
Q

What does ‘CD’ stand for as a monoclonal antibody precurser?

A

‘cluster of differentiation’. monoclonal antibodies have been developed that react with lineage specific lymphoid and myeloid activation and differentiation antigens

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17
Q

What does cytogenetics measure?

A

marrow cells are grown in a culture and analyzed for chromosome and structure, deletions, translocations, etc.

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18
Q

Childhood ALL, what percentage B cell vs T cell?

A

B cell 85%, T cell 15%.

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19
Q

Which subtype of ALL is associated with very high WBC and mediastinal mass? Who is it most commonly affecting?

A

T cell. more common in adolescent boys.

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20
Q

What is Mature B Cell?

A

Burkitt. Responds poorly to ALL thereapy, but do well when treated with Burkitt lymphoma therapy

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21
Q

Which is generally favorable, hyperdiploidy or hypodiploidy?

A

Hyperdiploidy (extra copies) is usually favorable.

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22
Q

Children with a presenting WBC higher than _______ generally have a poorer diagnosis

A

50,000

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23
Q 
Favorable or unfavorable:
TEL-AML?
Mixed Lineage Leuk (MLL)
BCR/ALB (phil chromosome)
Trisomy 4,10
A

t(12;21): associated with the TEL-AML1 gene product and conferring a very favorable prognosis; this translocation is most common in B precursor ALL and is associated with >90% chance of long-term survival
t(4;11): associated with the MLL gene product and a very poor prognosis, most often seen in infant ALL
t(9;22): associated with the BCR/ABL gene product and known as the “Philadelphia chromosome (Ph+)”
This type of ALL responds poorly to standard therapy and historically has been associated with poor outcomes. However, recent advances in targeted therapy appear to be improving the outlook for these patients.
Trisomy (an extra copy of the entire chromosome) of chromosomes 4 and 10 is associated with an excellent outcome in childhood ALL.

24
Q

Favorable age at diagnosis in ALL?

A

Between the ages of 2-10. Infants usually do the worst.

25
What are the ratings for marrow response to therapy? What do ALL children have at diagnosis?
“M” is for marrow “1” is for remission marrow (<5% blasts) “2” is for partial response (5% to 25% blasts) “3” is for frank leukemia (>25% blasts) All children with ALL have M3 marrow at diagnosis. After 1 to 2 weeks of induction therapy, most children have M1 or M2 marrow. (M3 marrow by this point in therapy is generally considered a slow early response.) More than 98% of children with ALL will have M1 marrow by the end of the first month of therapy.
26
Typical induction therapy has these 4 drug types
1- Glucocorticoid 2- Vincristine 3 - Asparaginase 4- Anthracycline
27
Typical consolidation therapy has which 7 components?
``` 1 - Cytarabine 2 - Mtx 3 - Etoposide 4 - Cyclophosphamide 5 - 6mp or 6tg 6 - VCR 7 - Asparaginase ```
28
Typical therapy in IM?
Mimics maintenance, but higher doses and more frequent. VCR, MTX and 6mp (capizzi vs high dose)
29
What is intensification typically like?
Usually mimics consolidation
30
How is treatment different for children who are CNS 2 or 3?
usually with more than 5 blasts is definitely positive (CNS 3) lower but questionable is CNS 2 and none is CNS 1 (neg). If they have symptoms of CNS involvement like nerve palsies, they are also considered positive regardless of blast result. They will get radiation in higher doses and IT chemo and high dose cytarabine
31
Cutoff for early vs late relapse
36 months from diagnosis
32
Possible side effects of leukostasis?
sluggish circulation due to elevated WBC count, can cause stroke, pulmonary infarct, etc.
33
What is the standard classification for AML?
WHO. (the FAB system has fallen out of favor, ex: M1, M2, M3, etc.)
34
What is AML with multilineage dysplagia?
AML that was previously MDS
35
Which are favorable vs unfavorable cytogenetic types of AML? 1) t8, 21 2) t15, 17 3) inv 16 4) 9, 11 5) FLT3 (FMS like tyrosine kynase) ITDs (internal tandem duplications) 6) GATA1 gene mutation 7) Monosomy 7
There are numerous structural changes with prognostic implications in childhood AML. Some of the most common are: t(8;21): associated with FAB M1 and M2 subtypes and conferring a favorable prognosis t(15;17): associated with M3 (promyelocytic) subtype and a good prognosis (if treated with appropriate therapy) inv(16) (inversion of chromosome 16): associated with M4 subtype and the best prognosis t(9;11), or any 11q23 rearrangements: associated with M4 and M5 subtypes and also seen in therapy-related AML and conferring a very poor prognosis Mutations of the FMS-like tyrosine kinase 3 (FLT3) gene involving internal tandem duplications (ITDs) are also associated with a poor prognosis. Mutations of the GATA1 gene are associated with M6 and M7 subtypes, specifically with Down syndrome. Prognosis is unknown at this time. Monosomy 7: associated with poor prognosis unless associated with Down syndrome
36
Favorable or unfavorable in AML at diagnosis? WBC above 100,000? Down syndrome? Treatment related etiology?
Children with AML who have WBC >100,000 at diagnosis generally have a poorer prognosis. The presence of Down syndrome usually indicates a more favorable prognosis. These children are treated on lower intensity protocols. Children with treatment-related AML (resulting from prior chemotherapy given for another cancer) generally have a dismal prognosis.
37
MPO positive, Sudan Black B positive cytochemistry usually means
AML
38
What drug of choice for APL?
all-trans retinoic acid (ATRA)
39
What are the therapy phases for AML?
1) Induction or remission (2 cycles) 2) Post Remission Intensified chemo (either chemo or HPCT) There is no maintenance phase except for APL, where they have 1-2 years maintenance. Usually 6-9 months, generally in hospital
40
What is treatment like for low risk AML patients? What is 'cell cycle intensive'
typically just chemo and no transplant. It is meant to manipulate the cell cycle by giving severely myelosuppressive chemo at short intervals (10 days and then 3-4 weeks recovery). Therapy is ADE - arac, dauno and etop. sometimes IT chemo is included
41
Relapsed AML treatment
mitoxantrone, asparaginase, fludarabine, clofarabine. HPCT if suitable donor.
42
Which leukemia can present with DIC and why
APL. characterized by granules in the blasts; these granules contain procoagulants, so when the blasts are destroyed it can cause DIC. usually gets worse when chemo is initiated.
43
What is ATRA?
Vit A derivative that helps the promyelocytes mature before they are destroyed in APL, which wouldn't cause the dramatic granule release that leads to DIC
44
Possible side effects of ATRA?
ATRA syndrome is a cardiorespiratory distress syndrome usually combined with increased WBC. Treat with steroids. Syndrome occurs in about 25% of APL patients and is fatal in 8-15%. Also can cause psedotumor cerebri.
45
What is pseudotumor cerebri?
an ATRA complication - increased ICP without infection. Treatmend aimed at reducing ICP with diuretics and serial LPs
46
Another possible APL med, other than chemo and ATRA, is
arsenic. often in relapsed, but studied in new as well
47
What % of APL patients have complete remission?
90%
48
Less intensive chemo is recommended for AML Down syndrome patients who are under what age? Otherwise what is the plan?
under age 4, toxicity risk is too much. over, treat same as other AML. Better prognosis with Downs, and if under age 2.
49
WBC above 200,000 can cause
leukostasis, sluggish circulation due to high WBC that can cause stroke, pulmonary infarct, etc. possible hydration, apheresis or exchange transfusions needed.
50
Is tumor lysis syndrome more common in ALL or AML?
ALL
51
Which patients are at increased risk of alpha strep infection?
AML - so they always need gram positive abx like vanc as well as broad-spectrum.
52
What is VRE?
Vanc resistant enterococcus. contact precs
53
3 phases of CML? How often in childhood?
Rare, 3% of childhood leuk. Natural course of illness is: 1) Chronic phase, usually about 3 years, they do well 2) Accelerated phase, splenomegaly, blasts, weakness, bruising, about 6 mo 3) Blast crisis, converts to acute form like AML or ALL and is usually fatal within 12 mo
54
Treatment for CML in chronic phase?
hydroxyurea or bisulfan, but usually don't cure. Tyrosine kinase inhibitors, like imatinib or dasatinib often achieve cytogenetic remission. long term efficacy is not known. Only proven cure is HPCT, but it is a tricky call if they respond to imatanib
55
What is JMML
very rare cancer only seen below 4yo. high monocyte count and high fetal hgb. Juvenile Myelomonocytic leukemia. Often fatal, generally get HPCT

CPHON Slides (24 decks)

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