Malignancy Flashcards

1
Q

What is the myeloperoxidase stain for on BM asp?

A

Shows up granulocytes and myelocytes yellow bits

Should be neg in ALL

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2
Q

What the esterase stain useful for on BMA?

A

Shows up monocytes red

Should be neg in ALL

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3
Q

?definition of acute leuk on BMA

A

> 20%blasts

😇Normal is less than 5%

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4
Q

?BMA FEATURES in ALL

A

Lymphoblasts, prob wall to wall
Monomorphic appearance

Blasts are usually small to med size, are larger than rcc, no cytoplasm
Cytoplasm they do have is blue-ish because agranular basophilic (high nuclear to cytoplasmic ratio, no granules
Chromatin “open” and is paler and less clumpy
May See vacuoles and nucleoli

Precursor Lymphoblasts will often have markers is has no place expressing (myeloid markers) - reflective of mutations

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5
Q

? What triggers ALL

A

Don’t really know - 2 crucial events
🍼Maturation arrest (stuck at blast phase)
👮🏾Loss of CONTROL of prolif

Some cytogenetic/molec predisp on Guthrie

More common in DS

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6
Q

?risk of ALL & AML in DS

A

DS 9-13% cases of AML
CURE rate higher with CHEMO alone (GATA-1)
10-30 x increased risk
10%will have a transient myeloproliferative disorder in newborn/early infancy
- high wcc and hepatosplenomegaly
- 70% spont remission, 30% progress to acute
Megakaryoblastic leuk at 1-3y

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7
Q

?subtypes ALL

A

Precursor B cell
T-cell (higher risk than pre-B ALL)
Mature B cell (Burkitts)
Directs therapy

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8
Q

What mutation is associated with TMD and AMKL in DS?

A

Mutations in GATA1 gene (5-10% of DS infants)
- found in BOTH TMD and AMKL

GATA 1 is a transcription factor crucial in erythro and megakaryopoiesis

30%TMD acquires other genetic abberations

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9
Q

?child cancer rate in NZ in

A
150 in 10 000 children per year
43%haem malig
💉💉💉Most leukaemia 34% (51 per 100 000)
- peak of leuk in preschoolers (1-4y)👦🏼
9%lymphoma (13 per 10 000/y)
21%CNS tumours 💭
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10
Q

?prognostic features in ALL

A

Tumour biology very NB, cure rate overall now 90%
Cytogenetics:
👼>50 good, Trisomy 4,10 good hyperdiploid)
See photo!
Pres wcc is not diagnostic in T-ALL (new)
But in prec B-cell high risk is pres wcc >50

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11
Q

How to assess chemo sensitivity in haem malig?

A

Minimal residual disease >0.01%

Not detected under 🔬

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12
Q

?cure rates in ALL (3-5y)

A

90%overall
95% for standard risk
99% for double/triple trisomies or ETV6-RUNX1

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13
Q

Treatment targeted to BCR-Abl1 gene (t(9;22)

A

Imatinib/dasatinib
Tyrosine kinase inhibition, combination therapy has negated need for transplant
ALL Ph positive Current 7 year relapse free survival >70%
Classic CML with Philadelphia chrom

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14
Q

Infant ALL ? Common cytogenetic feature

Prognosis

A

Different kettle of fish, rare, most are MLL rearrangements and often have high presentation wcc
Much poorer outcomes, best case scenario 50/50 cure rate

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15
Q

?differences between ALL and AML chemo

A

ALL 3years fairly light chemo

AML 6months heavy duty and cytarabine based

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16
Q

AML ?cure rate

A
Second most common leu in childhood (less than ALL)
80-90% remission
50-60% disease free survival
Inc incr with age
5y 60-65% OS
17
Q

Key AML pres features

A

CNS 10%
BM and immune phenotype different to ALL: CD 13, 33
Masses or skin lesion (chloroma) extra medullary haematopoeisis
Gum hypertrophy
Others vague - wt loss, bruising, pancytopenia, bleeding.
NEED TO WATCH OUT FOR DIC
-thrombocytopenia
red cell fragments ALWAYS BAD (HUS, TTP, mech heart valve)
DS has x20 higher risk of leukaemia

18
Q

AML ?features on blood film

A

Auer rods (always in the exam!)
Blasts larger with granules in cytoplasm
Chromatin is pale and more open, still have vacuoli

19
Q

Classification AML

A

FAB 0-7 based on morph and histochem characteristics

WHO gives prognosis - based on morph, immunophenotype, and cytogenetic features

20
Q

Acute promyelocytic leu

A

Bundles of Auer rods
May present with DIC, FAB M 3 does pred prog
Induction chemo with ATRA (unique, good prof if doesn’t die during induction, can get away without cytotoxic)

21
Q

Reed Sternberg cells

A

Owls eye = hodgkins
Pathognomic and malignant
Often chronic course, may filter through surgical depts first, may get false FNAs (need and excision biopsy to capture)
Commoner in 2nd decade, more in females except when

22
Q

Features HOdgkin

A
May be prolonged hx
May be med mass
Lymphadenopathy progressive
Paraneoplastic phenom may be present (B sx, wt loss >10% over 6m, fever, drenching night sweats)
Ann arbour staging
23
Q

Mx of HL

A

PET may upstage or downstage disease or may allow avoidance further Radiotherapy

24
Q

ALL signs and symptoms

A
Leukaemic infiltrate in bone marrow, pancytopaenia
Anaemia, fever, bruising
HSM, adenopathy
Malaise, anorexia
Peak incidence 2-5y preschool peak
25
Q

ALL induction (to induce remission)

A
Around 28-30 days
1) pred as single agent first week
2) vincristine, Daunorubicin, asparaginase
Intrathecal MTX
98% remission rate after!
26
Q

Who requires radiation in ALL?

A

CNS INV
inadequate Rx response
T CELL ALL, high WCC

27
Q
ALL Total duration 
Consolidation
CNS
reinduction
Maintenance
A

24 months total
Maint part is 18m
ANZCHOG study 9 from Dec 2011
Nearly 90% cure

28
Q

Indications for HSCT in ALL

A
Offered to persistently high MRD at end of induction
Poor CYTOGENETICS 
Poor therapy response:
Poor pred response failure to ach CR1
High flow day 15
High MRD
29
Q

AML important clinical factors

What 2 parameters are predictive of outcome?

A
Infants now doing well 60-70%DFS if FAB M4/5 t(9;11) translocation
Prognosis worse as age increases (>10y)
CNS pos at dx not adverse
1) response to initial therapy
2) CYTOGENETICS 
Fav: t(8;21), t(5;17), inv 16
Fab 3
Adverse: (survival 17% at 8y)
monopsony 5/7
Del long arm chrom 5
Abnorm long arm chrom 3
Complex karyotype >4abnorms
30
Q

Role of HSCT in AML

A

AML CR2 and beyond
Refractory
High risk patients CR1
-should have MSD or alt donor transpl

Intermediate risk patients who had HSCT DFS 50% vs 39% so should have MSD if available

Low risk patients no transplant in CR1 (inv 16, t(8,21), trisomy 21

31
Q

What is the Immunophenotype of APL (acute promyelocytic leukaemia)

A

t(15;17) M3
ATRA induces differentiation
FAVOURABLE PROGNOSIS