18) Introduction to leukemias Flashcards

1
Q

2 characteristics that distinguish benign from malignant tumors

A

metastasis
differentiation

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2
Q

benign neoplasm that will mutate into a malignant neoplasm

A

premalignant

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3
Q

types of malignant neoplasms in BM

A
  • leukemia (BM and PB)
  • aleukemia leukemia (BM, not PB)
  • lymphoma (LN)
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4
Q

cell stages that mutations usually occur in

A
  • committed progenitor (myeloid or lymphoid)
  • pluripotential stem cell
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5
Q

inactivated/unaltered potential cancer-causing genes

A

proto-oncogenes

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6
Q

oncogenes result in…

A

unregulated cell proliferation without differentiation

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7
Q

body’s defense against oncogenes
function
potential problem

A
  • tumor suppressor gene (TSG)—p53 gene
  • suppresses cell proliferation and neoplastic transformation
  • homozygous mutation results in tumor growth
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8
Q

over 50% of cancers have —— gene suppression

A

p53/TSG

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9
Q

evidence for theory that it takes multiple mutations to cause a cancer

A

patients with other mutations have a higher risk of developing acute leukemia

  • Down syndrome
  • MPD or MDS
  • chronic leukemia
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10
Q

affect activation of oncogenes

A
  • genetic susceptibility
  • somatic mutation (radiation, benzene, drugs)
  • viral infection (especially retroviruses)
  • immunosuppression (hereditary, drugs)
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11
Q

3 general categories of heme neoplasms

A
  • myeloproliferative disorders (MPD)
  • myelodysplastic syndromes (MDS)
  • acute leukemias
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12
Q

2 classifications of leukemias

A
  • FAB (French American British) 1976
  • WHO 2016
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13
Q

the FAB system is based on…

A
  • blast morphology
  • cytochemical stains
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14
Q

leukemic hiatus

A

we see many blasts and mature cells, but few in-between states

characteristic of acute leukemia

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15
Q

all AML arise from…

A

CFU-GEMM

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16
Q

2 different FAB ways to subtype ALL

A

L1
L2
L3

or

T-cell
B-cell
Null cell

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17
Q

categories of ALL based on morphology & heterogeneity of BM blasts

A
  • L1: homogeneous
  • L2: heterogeneous
  • L3: Burkitt’s lymphoma (vacuolated lymphs)
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18
Q

M0 - M7 are subtypes of…

A

AML

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19
Q

neoplastic cell in MPD and MDS

A

pluripotential SC

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20
Q

subgroups of MPN

A
  • CML (↑ grans)
  • polycythemia vera (↑ rbcs)
  • essential thrombocythemia (↑ plts)
  • agnogenic myeloid metaplasia/myelofibrosis (↑ fibrotic marrow)
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21
Q

subgroups of MDS

A
  • refractory anemias (RA, RARS, RAEB, RAEB-T)
  • CMML (like CML + monos)
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22
Q

exception to characteristics of an MDS

A

CMML
↑ grans and monos in PB

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23
Q

WHO classification is based on…

A
  • cell lineage
  • morphology
  • immunophenotyping
  • genetic features
  • clinical syndrome
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24
Q

6 major myeloid groups based on WHO criteria

A
  1. MPN
  2. MDS/MPN
  3. MDS
  4. AML
  5. Acute leukemia of ambiguous lineage
  6. Myeloid & lymphoid neoplasms with eos and rearrangement
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25
Q

5 major lymphoid groups based on WHO criteria

A
  1. Precursor lymphoid neoplasms (ALL)
  2. Mature B-cell neoplasms (CLL)
  3. Mature T-cell and NK neoplasms
  4. Hodgkin’s lymphoma
  5. Posttransplant lymphoproliferative disorders
26
Q

peak leukemia incidence ages

A

2-5 years
> 50 years

27
Q

4 types of leukemia typical age groups

A

ALL: 2-5 years
CLL: adults >50 years
AML: adults, some children
CML: adults, middle age to older

28
Q

typical leukemia findings on presentation

A
  • anemia
  • thrombocytopenia
  • neutropenia/recurring infections
  • bone pain
  • hepatosplenomegaly, lymphadenopathy (chronic)
29
Q

examples of where heme neoplasms can infiltrate body

A
  • skin (especially those involving monos)
  • CNS (espeically ALL)
30
Q

green tumor of immature WBCs resulting from MPO activity

A

chloroma

31
Q

chloromas especially found in —— and ——

A

AML
CML

32
Q

anemias related to heme neoplasms are usually ——-/——-

A

normo/normo

33
Q

auer rods are found in ——— and ——– cells

most commonly in…

presence rules out…

A

blasts and pros
M1 and M2 AML
ALL

34
Q

————– erythropoiesis may be present in BM during leukemia, and does not respond to…

A

megaloblastoid
B12/folate tx

35
Q

chemical signs of rapid cell turnover in leukemia

A

↑ uric acid (product of nucleic acid metabolism)
↑ serum LDH (correlates with concentration of leukemic cells)

36
Q

now a last resort diagnostic method

A

cytochemical stains

not used at all for chronic leukemias

37
Q

number and type of surface markers can define… (3)

A
  • lineage
  • stage of development
  • clonality
38
Q

immunophenotyping is most useful in the dx of…

A

ALL

39
Q

t(15;17)

A

acute promyelocytic leukemia

40
Q

t(9;22)

A

CML (Philadelphia chromosome)

41
Q

used to catch the 5% of CML patients without Philadelphia chromosome

A

molecular methods that detect BCR/ABL mutation

42
Q

abnormal hormone receptor found in acute promyelocytic leukemia

A

retinoid acid receptor alpha (RARA)

43
Q

molecular analysis is most helpful in dx of … (3)

A
  • acute promyelocytic leukemia
  • CML
  • T & B cell ALLs
44
Q

—— of children treated for ALL go into long-term remission

adults treated for ALL have a ——- year survival rate

A

> 90%
2 year

45
Q

assess statistical data to develop protocols for leukemia tx

A

cooperative oncology groups
cancer and leukemia groups

46
Q

definition of minimal residual disease

A
  1. absence of leukemic blasts in BM and PB
  2. negative cytogenetics
  3. positive molecular tests (PCR, FISH)
47
Q

chemo drugs typically used and action

A
  • antimetabolites (kill rapidly dividing cells)
  • alkylating agents (kill resting & proliferating cells)
  • antibiotics (interfere with cell replication)
48
Q

3 phases of ALL tx

A
  • induction (achieves remission)
  • CNS prophylaxis
  • maintenance chemo (2-3 years in children)
49
Q

AML tx is the same as ALL, except…

A

no CNS prophylaxis

50
Q

permanent remission is rare and treatment is conservative

A

CLL

51
Q

few are cured, so tx is supportive

A

MPN and MDS

52
Q

BM transplant most successful in patients…

A

< 40 years

53
Q

treatment before BM transplant

A

chemo & irradiation until remission is achieved

54
Q

can be used if there is no well-matched BM donor found

A

autologous donation

55
Q

autologous donations must be collected when……

and treated with…

A

patient in remission
mAb to remove leukemic cells
cyropreserved

56
Q

—— days after a SC transplant, new cells begin to be produced

A

10-21

57
Q

synthetic GFs that can be used in tx

A
  • EPO
  • G-CSF
  • GM-CSF
  • IL-11 (megakaryos)
58
Q

chemo as a procoagulant can precipitate ——–, especially in patients with…

A

DIC
acute promyelocytic leukemia (pro granules are potent coag cascade activators)

59
Q

standard complication of chemo that must be monitored

A

pancytopenia—infection, anemia, bleeding

60
Q

supportive therapy used in addition to chemo

A
  • transfusions
  • antimicrobials