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Blood and Lymph Unit III > Acute Leukemias > Flashcards

Acute Leukemias Flashcards

1
Q

A clonal, neoplastic proliferation of hematopoietic cells, usually immature, presenting as a rapidly progressive disease =

A

leukemia

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2
Q

leukemic cells resemble cells of one or more myeloid lineages

A

acute myeloid leukemia

AML

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3
Q

leukemic cells resemble precursor (immature) lymphocytes

A

acute lymphoblastic leukemia

ALL

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4
Q

Abnormalities in Acute Leukemias …
Usually have___
May contain ___

A

Usually have chromosomal abnormalities - detectable by cytogenetic test

May contain more subtle abnormalities requiring molecular tests (e.g. pcr) for detection

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5
Q

Usually, abnormalities required to generate an acute leukemia include:

A

block in ability to differentiate

increased autonomy of growth-signaling pathways

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6
Q

Risk factors for acute leukemia? Two big ones? Plus others…(3)

A

Previous chemotherapy
Previous exposure of active marrow to ionizing radiation

tobacco smoke
benzene exposure
genetics

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7
Q

chemotherapeutic agents that can predispose to acute leukemia?

A

DNA alkylating agents

topoisomerase ii inhibitors

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8
Q

genetic syndromes that can predispose to acute leukemia?

A

down syndrome
bloom syndrome
fanconi anemia
ataxia telangiectasia

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9
Q

Clinical presentation of acute leukemia (3)

A

signs/symptoms of anemia

signs/symptoms of thrombocytopenia

signs/symptoms of neutropenia

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10
Q

signs and symptoms of anemia?

A

fatigue, malaise, pallor, dyspnea

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11
Q

signs and symptoms of thrombocytopenia

A

bruising, petechiae, hemorrhage

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12
Q

signs / symptoms of neutropenia

A

fever / infections

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13
Q

Clinical presentation of acute leukemia - effects directly attributable to leukemic cells?

A

Thrombotic events due to increased blood viscosity (leukostasis)

Disseminated intravascular coagulation

Direct infiltration of skin, gums, lymph nodes, and/or other tissues by leukemic cells

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14
Q

what is leukostasis

A

increased blood viscosity - in setting of leukemia has very high WBC count

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15
Q

Disseminated Intravascular Coagulation - is initiated by?

A

the leukemic cells in some types of AML (acute myeloid leukemia)

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16
Q

Acute lymphoblastic leukemia - neoplasms of?

A

neoplasms of precursor lymphoid cells

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17
Q

neoplasms of precursor lymphoid cells predominately manifest as leukemia (ALL); much less commonly they may manifest as?

A

a solid mass - referred to as lymphoblastic lymphoma

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18
Q

2 types of ALL

A

B-lymphoblastic leukemia

T-lymphoblastic leukemia

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19
Q

ALL incidence?

A

1-5/100,000/yr

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20
Q

Age demographic of ALL?

A

75% of cases of ALL occur in children less than 6 years old

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21
Q

Is there a set percentage of blasts required to diagnose ALL?

A

No! ALL patients nearly always present with blasts representing a majority of marrow cells (“packed marrow”); thus, there is not set percentage

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22
Q

What does the peripheral blood white count look like in ALL?

A

May be markedly increased, mildly increased, normal, or decreased

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23
Q

How do we determine blast type (myeloblast vs. lymphoblast; B-lymphoblast vs T-lymphoblast) in ALL?

A

Requires immunophenotyping

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24
Q

Which marker should you know is a generic marker of immaturity (seen on both lymphoblasts and myeloblasts?)

A

CD34

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25
Q

What is a common lymphoblast marker? (not on mature lymphocytes)

A

TdT

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26
Q

What are markers of B cell lineage to know?

A

CD19

CD22

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27
Q

What are markers of T cell lineage to know?

A

CD3

CD7

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28
Q

The majority of ALL cases are?

A

B-lymphoblastic leukemia (80-85%) Thus, we would see CD19/CD22

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29
Q

In addition to expressing CD19/CD22, what else would we expect from B-ALL cell surface expression?

A

The absence of CD20 - which is a mature B cell surface immunoglobulin

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30
Q

Which type of ALL do we typically see in childhood?

A

B-ALL

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31
Q

What does it mean for B-ALL to have the t(9;22); BCR-ABL1 cytogenetic finding?

A

t(9;22) results in a derivative chromosome 22, the so-called Philadelphia chromosome, encoding the BCR-ABL fusion tyrosine kinase protein (thus, these cases are often called “Ph+ALL”)

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32
Q

t(9;22) frequency in adult and childhood ALL?

A

25% adult

2 % childhood

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33
Q

What is different between t(9;22) in ALL vs. CML

A

Fusion protein differs from the BCR-ABL fusion protein in CML, as it is only 190kD (p190), due to its resulting from a different breakpoint in BCR than the typical CML breakpoint

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34
Q

p190?

A

The 190kD t(9;22) fusion protein in B-ALL (BCR-ABL)

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35
Q

If you have B-ALL, would you want to have t(9;22)?

A

No! The presence of t(9;22) in ALL is an unfavorable prognostic factor

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36
Q

Tell me about B-ALL with translocations of 11q23; (MLL)

partner genes?
demographic?
prognosis?

A

MLL may be rearranged with any of multiple possible partner genes

Frequently seen in neonates and young infants

Poor prognosis

37
Q

Tell me about B-ALL with t(12;21); ETV6-RUNX1

Demographic?
Prognosis

A

25% of cases of childhood

Very favorable prognosis

38
Q

What percent of ALL cases are accounted for by T-ALL

A

25%

39
Q

When compared to B-ALL, does T-ALL occur more or less frequently in young adults/adolescents

A

T-ALL more frequently occurs in adolescents and young adults

40
Q

When compared to B-ALL, does T-ALL more or less frequently present with component of lymphoma?

A

more frequently presents with a component of T-lymphoblastic lymphoma (T-LBL), often present as a mediastinal mass

41
Q

Does B-ALL or T-ALL present more commonly with an elevated WBC count?

A

T-ALL

42
Q

T-ALL males vs females?

A

T-ALL favors males over females

43
Q

ALL

Prognosis in Children and Adults?

A

“good prognosis disease” in children with complete remission rates >95% / cure rates 80%

In adults, the prognosis for ALL is worse than children, with complete remission rates of 60-80% cure rates of

44
Q

In ALL, for the best prognosis, how old would you want to be? How old would you not want to be?

A

You would want to be 1-10 for the best prognosis

You would not want to be an infant (

45
Q

In ALL, would you rather be B-ALL or T-ALL

A

You would want to be B-lymphoblastic - it has better prognosis

46
Q

In ALL, what kind of diploidy would you want?

A

Hyperdiploidy (51-65)

you would not want hypodiploidy (

47
Q

Would you be happy or sad to hear that you had a high WBC in ALL?

A

Sad… associated with worse prognosis

48
Q

Would you be happy or sad to have your tumor respond slowly to treatment?

A

sad… worse prognosis

49
Q

Is AML or ALL more heterogenous?

A

AML (more types)

50
Q

AML age demographic?

A

AML is typically disease of adults

- average age at dx = 65

51
Q

What % childhood leukemia AML?

A

10

52
Q

AML incidence?

A

3/100,000/yr

similar to ALL

53
Q

What is the diagnosis of AML usually associated wtih?

A

presence of 20% or greater myeloblasts in the marrow and/or peripheral blood

54
Q

What are three ways to determine the myeloblast percent in the marrow / blood

A
  1. morphological appearance while performing diff count on marrow aspirate or peripheral blood
  2. flow cytometric analysis of marrow aspirate or peripheral blood
  3. immunochemistry performed on marrow core biopsy
55
Q

AML

Generic marker of immaturity (also on lymphoblast) we woudl expect to see?

A

CD34

56
Q

AML

Common myeloid markers we would expect to see (not usually on lymphoblasts)

A

CD117 (C-Kit)

Myeloperoxidase

57
Q

Myeloblast morphology (vs. lymphoblast) and diagnosis?

A

Myeloblasts typically have the generic appearance of a blast, and cannot be definitely differentiated from lymphoblasts based on morphology alone…
In some cases of AML (or MDS), some of the myeloblasts may contain Auer rods, allowing for their identification as meyloblasts by morphology alone?

58
Q

What do auer rods tell us?

A

AML

59
Q

Which cytogenetic abnormalities allow a diagnosis of AML despite regardless of blast count? (5)

A 
t(8;21) RUNX1-RUNX1T1
inv(16) or t(16;16); CBFB-MYH11
t(15;17); PML-RARA
t(1;22); RBM15-MKL1
11q23; MLL
60
Q

AML with t(8;21); RUNX1-RUNX1T1

percentage of AML cases?
age demographic?
characteristic?
prognosis?

A

5% AML cases
younger patients
associated wtih “AML with maturation”
relatively good prognosis

presence of translocation is diagnostic of AML regardless of blast count

61
Q

what is RUNX1?

A

(also rearranged in some cases of ALL) encodes the alpha subunit of core binding factor (CBF), a TF needed for differentiation

62
Q

What is AML with maturation?

A

some mature neutrophil production is still occuring

63
Q

AML with inv(16) or t(16;16); CBFB-MYH11

prognosis?
associated with?

A

Relatively good prognosis

associated with the presence of abnormal eosinophilic precursors with abnormal basophilic granules in addition to eosinophilic granules (“baso-eos”)

typically have myelomonocytic leukemia (leukemia cells are a mixture of myeloblasts and monocytes)

*5-10% AML cases
younger patients
presence of this translocation is diagnostic of AML regardless of blast count

64
Q

What is CBFB?

A

encodes beta subunit of core binding factor (CBF) - a TF needed for differentiation

65
Q

baso-eos are associated with which cytogenetic finding in CML?

A

inv(16)

t(16;16) CBFB-MYH11

66
Q

Myelomonocytic leukemia

A

leukemic cells are mixture of myeloblasts and monocytes

seen in AML with inv(16) or t(16;16) CBFB-MYH11

67
Q

AML with t(15;17); PML-RARA

aka

A

Acute promyelocytic leukemia (APL)

68
Q

What is acute promyelocytic leukemia? (APL)

A

leukemic cells are blocked at the promyelocyte stage (Rather than blast stage)

seen in AML with t(15;17) PML-RARA

typically, cells have hypergranular morphology, resembling normal promyelocytes

may see with multiple auer rods

presence of translocation is diagnostic of AML regardless of blast/promyelocyte count

69
Q

Hypergranular promyelocytes are characteristic of?

A

Acute promyelocytic leukemia

70
Q

Promyelocytes with multiple auer rods are characteristic of?

A

Acute promyelocytic leukemia

71
Q

2 reasons why recognition of APL is important?

A
  1. The RARA gene encodes the retinoic acid receptor alpha protein. Signalling through this receptor is required for differentiation past the promyelocyte stage (treat with all trans retinoic acid (ATRA))
  2. APL is often associated with DIC
72
Q

Do patients with APL require traditional induction chemo?

A

No! This is why it is important to recognize PML-RARA t(15;17) because patients can be treated with ATRA and arsenic salts, which have very limited morbidity and almost no mortality (unlike chemo)

73
Q

AML with t(1;22) RBM15-MKL1

characteristics
demographics
prognosis

A

usually shows megakaryoblastic differentiation

more often seen in infants with down syndrome

relatively good prognosis (in relation to other infantile leukemias)

74
Q

AML with abnormalities of 11q23; MLL

Characteristic gene partners?
phenotype
prognosis

A

MLL may be rearranged with multiple possible partner genes

AML with abnormalities of MLL frequently shows some degree of monocytic differentiation

poor prognosis (similar to cases of ALL with abnormalities of MLL)

75
Q

What is MLL anyway?

A

Histone methyltransferase that plays an essential role in early development and hematopoiesis.

76
Q

Therapy related AML (t-AML)
definition?
percent account?
prognosis?

A

t-AML is defined as AML arising secondary to DNA damage from a prior therapy

t-AML accounts for 10-20% of AML

very bad prognosis

77
Q 
t-AML
Alkylating agents / radiation 
- latency period from therapy to AML or MDS?
- Typical cytogenetic abnormality?
- Progesses to AML through MDS?
A
  • latency 2-8 yr
  • complex karyotype with whole or partial deletions of 5&7
  • usually through MDS
78
Q

t-AML
topoisomerase inhibitors

  • latency period from therapy to AML or MDS?
  • Typical cytogenetic abnormality?
  • Progesses to AML through MDS?
A

latency - 1-2 years

  • rearrangements of 11q23; MLL
  • usually not through MDS
79
Q

What do cases of AML NOS include?

A

cases of AML lacking recurrent cytogenetic findings, and not know to be due to previous therapy

*but may be subclassified based on the line of differentiation of the leukemic cells, as defined by morph and immunophenotyping

80
Q

In undifferentiated / minimally differentiated / or with maturation AML, what are the leukemic cells?

A

myeloblasts

81
Q

In myelomonocytic AML what are the leukemic cells

A

myeloblasts and monocytes

82
Q

In monoblastic or monocytic AML, what are the leukemic cells?

A

monoblasts,
promonocytes,
and/or monocytes

83
Q

In monoblastic or monocytic AML, what clinical manifestation should you be aware of?

A

leukemic cells often cause lesions in skin and gums

84
Q

In megakaryoblastic AML, which leukemic cells are present? what clinical things should you know?

A

megakaryblasts

often associated with significant marrow fibrosis

85
Q

Molecular abnormality = FLT3 internal tandem duplication…

prognosis? (AML)

A

POOR

86
Q

Molecular abnormality = Nucleophosmin-1 (NPM1) mutation

prognosis? (AML)

A

GOOD if negative for FLT3 ITD

87
Q

CEBPA Mutation in AML

prognosis?

A

GOOD if negative for FLT3 ITD

88
Q

AML approximate complete remission rate?

A

60%

89
Q

AML therapy of choice for younger patients, patients with high risk disease, and patients with relapsed disease?

A

Hematopoietic stem cell transplant

SCT

Blood and Lymph Unit III (3 decks)

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