Unit 2: Myeloid Leukemias Flashcards

1
Q

Most common type of adult leukemia (incidence increases with age)

A

Acute Myeloid Leukemia

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2
Q

True or false:

All myeloid leukemias are rapidly fatal if left untreated.

A

true

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3
Q

Acute myeloid leukemia involves a defect of __________.

A

HSC (CD34+)

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4
Q

In some AMLs you can find Auer rods = condensed to primary granules. This means cells are…

SBB and MPO+ in

A

myeloblasts or promyelocytes

SBB/MPO + (in mature myelocytes)

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5
Q

What are the two Non-specific Esterase stains (NSEs) – for monocytes?

A
  1. α-Naphthyl Butyrate Esterase (NBE)
  2. α-Naphthyl Acetate Esterase (NAE)
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6
Q

Specific Esterase Stains – stains for myeloid
cytoplasmic granules…used to separate monocyte
precursors from granulocyte precursors

A

Napthol AS-D Chloroacetate Esterase (NASD)

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7
Q

Diagnosis of acute myeloid leukemia begins with

A

Peripheral blood and bone marrow examination

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8
Q

WBC count could be variable in AML but most range from…

A

1 x 109/L - 200 x109/L

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9
Q

AML:

myeloblasts present in ___% of patients

A

90

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10
Q

Clinical presentation with AML?

A
  • Anemia
  • Neutropenia
  • Thrombocytopenia
  • Possible splenomegaly
  • Bone marrow is hypercellular with >20% blasts
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11
Q

What AML clinical presentations lead to pallor, fatigue, bruising, bleeding, and fever?

A
  • Neutropenia
  • Thrombocytopenia
  • Possible splenomegaly
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12
Q

AML Lab Findings:

_____________ due to increased cell turnover

A

Hyperuricemia

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13
Q

AML Lab Findings:

_____________ due to cell lysis

A

Hyperphosphatemia

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14
Q

AML Lab Findings:

__________________ due to progressive bone destruction.

A

Hypokalcemia

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15
Q

Hyperuricemia, Hyperphosphatemia, and Hypocalcemia are signs of what syndrome?

A

Tumor Lysis Syndrome – a group of metabolic
complications that can occur in patients with a malignancy

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16
Q

Generalizations for AMLs:

  • Many of these are due to mutations activating inappropriate __________ factors
  • All of these exhibit variable degrees of dysmyelo-poiesis
    (along with all of their other morphological abnormalities!)
A

transcription

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17
Q

abnormal hyperproliferation of bizarre
granulocyte &/or monocyte precursors

A

Dysmyelopoiesis

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18
Q

FAB Classification for AMLs:

Acute Myeloblastic (with Minimal Differen-
tiation) (of nucleus)

A

Mo

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19
Q

FAB Classification for AMLs:

Acute Myeloblastic w/o Maturation (of cytoplasm)

A

M1

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20
Q

FAB Classification for AMLs:

Acute Myeloblastic with Maturation (of cytoplasm)

A

M2

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21
Q

FAB Classification for AMLs:

Acute Promyelocytic

A

M3

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22
Q

FAB Classification for AMLs:

Acute Myelomonocytic

A

M4

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23
Q

FAB Classification for AMLs:

Acute Myelomonocytic w/Eosinophilic variant

A

M4(Eo)

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24
Q

FAB Classification for AMLs:

Acute Monocytic

A

M5

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25
Q

FAB Classification for AMLs:

Poorly differentiated =

A

M5a

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26
Q

> 20% blast count not
required if __________
found in AML

A

genetic mutations

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27
Q

Makes up 5% of AMLs; found in children and adults
younger than 60 yo

A

AML with t(8;21)(q22;q22)

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28
Q

t(8;21) = ______ prognosis

A

good

[ “It’s 2 good 2 have t(8;21).” ]

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29
Q

AML with t(8;21)(q22;q22):

WBCs have what functional problems?

A

with phago-cytosis &
chemotaxis

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30
Q

AML with t(8;21)(q22;q22):

Lab findings?

A

Blasts are large with abundant, dysplastic cytoplasm and numerous primary granules and Auer rods. Sometimes exhibit pseudo-Pelger-Huet.

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31
Q

AML with t(8;21)(q22;q22):

  • MPO & SBB _____
  • PAS ______
A

1+

negative

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32
Q

AML with t(8;21)(q22;q22):

SE ___
____ Auer rods

A

+

+/-

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33
Q
  • Rare: 5-8% of all AMLs and occurs at any age (usually young)
  • Myeloblasts, monoblasts, and promyelocytes observed
  • Also eosinophila with dyplastic changes
A

AML with inv(16)(p13.1p22)

34
Q

AML with inv(16)(p13.1p22):

Remission rate is good but only _____ are cured

A

half

35
Q
  • Makes up 8% of AMLs
  • Characterized by a differentiation block in the promyelocyte stage
A

AML with t(15;17)(q22;q12) – Acute Promyelocytic Leukemia (APL)

36
Q

Abnormal hypergranular promyelocytes with Auer rods
* Granules can obscure nucleus with abundant cytoplasm & variable
nuclear size & shape.

A

AML with t(15;17)(q22;q12) – Acute Promyelocytic Leukemia (APL)

37
Q

AML with t(15;17)(q22;q12) – Acute Promyelocytic Leukemia (APL):

Leukemic cells also have defective retinoic acid receptor (RARα gene), so can be treated with…

A

TRA (All Trans Retinoic
Acid, a vit. A derivative)

38
Q

How ATRA treatment work?

A

pushes pros into finishing maturation, thus causing
remission! (Then attacked with chemo. – a very successful combination.)

39
Q

Acute Promyelocytic Leukemia (APL) is strongly associated with _______.

A

DIC

-A promyelocyte’s primary granules are rich in
thromboplastin - like substances . . .which trigger a “bleeding diathesis”.

40
Q

What is the variant subtype of APL?

A

microgranular APL (cells look agranular if only light
microscopy used)

41
Q

Lab Findings FOR BOTH APL VARIANTS:

MPO & SBB =
PAS ____

A

2+

negative

42
Q

Lab Findings FOR BOTH APL VARIANTS:

SE ____
Both NSE _____

A

positive

negative

43
Q

Lab Findings FOR BOTH APL VARIANTS:

  • Frequent Auer rods, can be in bundles (so-called “faggot
    cells”).
  • ↓ plt. count because…
A

Plts. used up making clots

44
Q

Acute Myelomonocytic Leukemia (aka. AMML) lab findings:

peripheral blood absolute monocytosis (>___%); many
circulating blasts, pros, immature granulocytes & monos!
* >20% monos AND >20% granulocytes

A

20

45
Q

Acute Myelomonocytic Leukemia (aka. AMML) lab findings:

-MPO and SBB still _____
-Both BSEs now start to be ______

A

positive

positive

46
Q

Acute Myelomonocytic Leukemia (aka. AMML) lab findings:

  • SE still _____
  • PAS still ______
    -_____ Auer rods
A

positive

negative

+/-

47
Q

Acute Myelomonocytic Leukemia (aka. AMML) lab findings:

CD…

A

CD 11b, CD 4, CD 36, and CD 64

48
Q

AMML clinical symptoms are the common ones, PLUS…

A
  • CNS & soft tissue involvement
  • Ex., gum hypertrophy & skin lesions
49
Q

Acute Monocytic Leukemia (aka. AMoL)

-Makes up _____% of AMLs
-When _____% leukemic cells are monocytic

A

5-8

> 80

50
Q

Acute Monocytic Leukemia (aka. AMoL):

Usually CD ___ and CD ___ pos. (“gold std.”)

A

4

64

51
Q

Acute Monocytic Leukemia (aka. AMoL) is usually t(____)

A

t(8;16)

52
Q

clinical symptoms of AMoL?

A

Similar to AMML (with CNS & soft tissue involvements.)

53
Q

Why is there Increased serum & urinary lysozyme with AMoL?

A

Bc. this enzyme found in greatest amount in monos

54
Q

With AMoL, bone marrow shows some granulocytes, but majority composition is monoblasts, promonocytes, & monocytes.
* <___% granulocytes

A

20

55
Q

AMoL:

MPO and SBB _________

A

+ / - (most frequently NEG.)

56
Q

Are there Auer rods with AMoL?

A

Very rare Auer rods (most frequently NEG.)

57
Q

AMoL:

PAS __________

A

positive

58
Q

AMoL:

Both NSEs ________ (NBE ___)

A

positive

3+

59
Q

Acute Erythroid leukemia
(AEL) only makes up ___% of AMLs.

A

3

-Can evolve further into AMLs

60
Q

Only AML with marked erythroid precursor hyperplasia!!

A

Acute Erythroid leukemia
(AEL)***

61
Q

Acute Erythroid leukemia
(AEL) is Frequently preceded by a ____________ syndrome.

A

myelodysplastic syndrome (MDS, aka. Refractory Anemia).

62
Q

Acute Erythroid leukemia
(AEL) lab findings:

bone marrow shows ____________ (Atlas pp. 178-79) = abnormal proliferation of bizarre RBC precursors, with reverse M:E ratio due to huge # of abnormal erythroblasts,
& even a few ringed sideroblasts.

A

dyserythropoiesis

63
Q

Peripheral blood findings with Acute Erythroid leukemia (AEL)?

A

nRBCs with mkd. aniso, poik. & schistocytosis (due to the dyserythropoiesis)

64
Q

Acute Erythroid leukemia
(AEL) Lab Findings:

  • Lots of myeloblasts
  • Some abnormal _____________
A

megakaryocytes

65
Q

Acute Erythroid leukemia
(AEL):

> ____% bizarre, multinucleated, megaloblastoid nRBCs!!
(with few ringed sideroblasts.)

A

50

66
Q

Acute Erythroid leukemia
(AEL):

MPO/SBB ______. Why?

A

negative, Defect here is mostly dyserythropoiesis, not so much dysmyelo-
poiesis.

67
Q

Acute Erythroid leukemia
(AEL):

  • Both NSEs _______
  • PAS __________
A

positive

positive

68
Q

Acute Erythroid leukemia
(AEL):

Early, abn. erythroblasts are PAS + in “granular” or
“blocky” pattern. Later, abnormal erythroblasts + in
“_______” pattern.

A

diffuse

69
Q

Acute Erythroid leukemia
(AEL):

Normal RBC precursors —> PAS ______

A

not PAS + at all!

70
Q

Rarest form of AML!! (Makes up < 1% of AMLs.)

A

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL)

71
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL) is frequently associated with ________ syndrome.

A

Down’s Syndrome

72
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL):

Common leukemia symptoms + “dry tap” upon bone marrow aspiration due to ____________.

What other leukemia has “dry tap”?

A

myelofibrosis

Hairy Cell Leukemia

73
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL)
* Lab Finding: bone marrow shows primarily…

A

dysmegakaryopoiesis: ↑ #s of
megakaryocyte precursors, including megakaryoblasts,
atypical megakaryocytes (with “blebbing”), & giant plts. (Atlas pp. 182-83)

74
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL):

Few myeloblasts can look…

A

lymphoid or undifferen-tiated.

75
Q

Does AMegL show dyserythropoises?

A

Little accompanying dyserythropoiesis

76
Q

AMegL can be associated with t(____) & t(____).

A

t(3;21) & t(1;22)

77
Q

AMegL:

AMP and SBB ______. Why?

A

negative, so few myeloid elements

78
Q

AMegL:

NBE _____ but NAE ______. (unique to megakaryo-blasts)!

A

negative, positive

79
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL):

PAS ________
Plt. peroxidase _____

A

positive
postive

80
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL):

CD 41, 42, 61 ______

A

+/-

81
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL):

May plt.-specific receptors, such as _______________ + (for fibrinogen)

A

GPIIb/IIIa receptor

82
Q

Acute Megakaryocytic/
Megakaryoblastic Leukemia (AMegL):

Acid phosphatase _____

What other leukemia is acid phosphatase +

A

positive

but this isn’t spe-cific for megakaryoblasts! In what
other specific leukemia was acid phos. stain pos.? —>

T-cell ALL