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IBI Test 3 > B3-038 Big Case: Acute Leukemia > Flashcards

B3-038 Big Case: Acute Leukemia Flashcards

1
Q

reactivation of hematopoiesis outside bone marrow due to insufficient bone marrow reserve

A

extramedullary hematopoiesis

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2
Q

sites of extramedullary hematopoiesis

A

liver
spleen
lymph nodes
soft tissue

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3
Q

bone marrow consists of what structures?

A

sinusoids
hematopoietic cells
fat

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4
Q

normal ratio of hematopoietic cells: fat in adult bone marrow

A

1:1 (50% cellular)

**can also vary with age, 100-age

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5
Q

normal ratio of myeloid:erythroid cells in adult bone marrow

A

3:1

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6
Q

thrombocyte maturation

A

megakaryocytes –> platelets

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7
Q

granulocyte maturation

A

myeloblast ——–> band neutrophil –> neutrophil

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8
Q

primary granules contained in neutrophils

A

myeloperoxidase
lysozyme
defensins
acid hydrolases
neutral proteases

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9
Q

secondary granules contained in neutrophils

A

lysozyme
plasminogen activator
histamine
collagenase
lactoferrin
alkaline phosphatase

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10
Q

involved in anti-parasitic function, allergic reactions, chronic inflammation

A

eosinophils

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11
Q

eosinophil granules

A

peroxidase

toxic to helmiths and protozoans:
major basic proteins
eosinophil cationic protein
eosinophils derived neurotoxin

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12
Q

involved in IgE mediated allergic reactions

A

basophils

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13
Q

basophil granules

A

histamine
tryptase
chondroitin sulfate

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14
Q

precursors of macrophages

A

monocytes

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15
Q

monocyte granules

A

lysosomal enzymes

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16
Q

express CD2, 3, 5,

A

T lymphocytes

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17
Q

express CD 19, 20, HLA-DR, surface Ig

A

B lymphocytes

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18
Q

T and B lymphocytes are defined by

A

rearrangement of TCR or Ig genes

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19
Q

occurs in antigenically stimulated B cells in both heavy and light chain hypervariable regions

A

somatic mutation

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20
Q

somatic mutation: cells with ____________ affinity for antigen survive

A

increased

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21
Q

Somatic mutation can be detected via

A

amplification/sequencing of V region and comparison with known germline V genes

**used for prognosis in some B-cell neoplasms

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22
Q

too little clotting causes

A

hemorrhagic disorders

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23
Q

too much clotting causes

A

thrombotic disorders

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24
Q

hemorrhagic disorders of primary hemostasis causes

A

mucosal/skin bleeds

petechiae, purpura, epistaxis

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25
Q

hemorrhagic disorders of secondary hemostasis cause

A

soft tissue or joint bleeds

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26
Q

thrombotic disorders cause

A

endothelial injury
stasis
hypercoagulability

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27
Q

myeloid neoplasms can be caused by [3]

A

myeloproliferative neoplasms
acute myeloid leukemia
myelodysplastic syndromes

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28
Q

lymphoid neoplasms

A

lymphoblastic
luekemia/lymphoma

mature B cell neoplasms
mature T cell neoplasms
Hodgkin Lymphoma

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29
Q

neoplasm derived from precursor hematopoietic cells with proliferation and accumulation of blast cells

A

acute leukemia

**proliferation without maturation

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30
Q

> 20% blast increase in the bone marrow

A

acute leukemia

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31
Q

if untreated, rapidly progressive and fatal in weeks to months

A

acute

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32
Q

primarily in immature cells (blasts)

A

acute

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33
Q

primarily in mature or maturing cells

A

chronic

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34
Q

if untreated, slowly progressive and fatal in months to years

A

chronic

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35
Q

primary presentation in bone marrow and blood

A

leukemia

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36
Q

can be derived from myeloid cells or lymphoid cells

A

leukemia

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37
Q

primary presentation in lymph nodes or other lymphoid tissues

A

lymphoma

38
Q

derived from lymphoid cells only

A

lymphoma

39
Q

main types of acute leukemias [3]

A

acute myeloid leukemia (AML)

acute lymphoblastic leukemia/lymphoma (ALL, LBL)
-B-lymphoblastic
-T-lymphoblastic

40
Q

incidence increases steadily throughout adult life

A

AML

41
Q

most common malignancy of childhood

A

ALL

**85% are B-ALL, 15% T-ALL

42
Q

age of onset demonstrates bimodal distribution, with greater incidence occurring in childhood and after 50

A

ALL/LBL

43
Q

immunodeficiency states are an etiologic association for

A

ALL, LBL

44
Q

alkylating agents, topoisomerase II inhibitors are etiologic associations for

A

AML

45
Q

stem cell hematologic disorders are etiologic association for

A

AML

**aplastic anemia, PNH, etc.

46
Q

etiologic associations for AML and ALL/LBL

A

ionizing radiation
trisomy 21
chromosomal instability syndromes

47
Q

clinical findings associated with acute leukemias

A

anemia
thromobocytopenia
neutropenia
hepatosplenomegaly

48
Q

clinical findings specific to ALL

A

bone pain
lymphadenopathy

49
Q

pathologic findings of blood in acute leukemias

A

anemia
neutropenia
thrombocytopenia
circulating blasts

50
Q

pathologic findings of bone marrow in acute leukemias

A

increased cellularity, increased blasts

51
Q
A

blast cells

52
Q

what test determines lineage for acute leukemias?

A

flow cytometry

53
Q

myeloperoxidase +
TdT-
CD34
CD13
CD33
CD117

A

AML

54
Q

TdT+
myeloperoxidase -
CD10
CD19

A

B-ALL

55
Q

TdT+
myeloperoxidase -
CD2
CD3
CD5

maybe CD4/8

A

T-ALL

56
Q

auer rods

A

AML

57
Q
A

auer rods

58
Q

goal is to produce complete remission

A

induction therapy

59
Q

goal is long term disease free survival

A

post induction therapy

**i think he also called this consolidation?

60
Q

targeted therapies are mostly

A

adjunctive

61
Q

favorable markers for AML prognosis

A

t(15;17) -acute promyelocytic leukemia
t(2;21), inv (16)

**indicate loss of maturation

62
Q

unfavorable markers for AML prognosis

A

> 60
WBC >100,000
prior myelodysplastic syndromes
therapy-related AML

63
Q

t(15;17)

A

Acute promyelocytic leukemia

64
Q

disrupts retinoic acid receptor (RAR-a) gene

A

Acute promyelocytic leukemia

65
Q

abnormal promyelocyte like cells with multiple auer rods

A

Acute promyelocytic leukemia

66
Q

frequently associated with DIC

A

Acute promyelocytic leukemia

67
Q

ATRA used for treatment

A

Acute promyelocytic leukemia

68
Q

Acute promyelocytic leukemia prognosis

A

favorable to intermediate

69
Q
A

Acute promyelocytic leukemia

70
Q

favorable features for ALL prognosis

A

precursor B-ALL
4-10 years old
<10,000 per uL
hyperdiploidy t(12;21)

71
Q

unfavorable features for ALL prognosis

A

precursor T-ALL
<2 or >10 y.o.
WBC>100,00 per uL
extensive organomegaly
t(4;11), t(9;22)

72
Q

response to therapy: residual disease after initial therapy

A

predicts outcome

73
Q

adult ALL has a [better/worse] prognosis than childhood ALL

A

worse

74
Q

t(4;11)

A

unfavorble for ALL

75
Q

t(9;22)

A

unfavorable for ALL

76
Q

hyperdiploidy t(12;21)

A

favorable for ALL

77
Q

t(2;21)

A

favorable for AML

78
Q

acute myeloid leukemia is characterized by expression of

A

myeloperoxidase
CD34
CD13
CD33
CD117

and/or presence of auer rods

79
Q

ALL is characterized by

A

T or B lymphoid markers
TdT+ expression

80
Q

the presence of ….. is diagnostic for AML

A

auer rods

81
Q

hepatosplenomegaly is an unfavorable prognostic for

A

ALL

82
Q

complex karyotype including the presence of 3 or more abnormalities is an unfavorable prognostic marker for

A

AML

83
Q

CNS involvement with seizures is more common in

A

ALL

84
Q

blasts with prominent granules are seen in

A

t(15;17) APL

85
Q

the diagnosis of AML requires

[2]

A

blasts with myeloperoxidase expression
20% blasts in bone marrow

circulating blasts may vary

86
Q

Auer rods are ….. but not……to diagnose AML

A

sufficient but not required

87
Q

elevated lymphocytes suggest the possibility of

A

CLL

88
Q

hypercellular bone marrow with <20% blasts

A

AML

89
Q

what are you likely to see on blood smear of APL

A

shistocytes due to high correlation with DIC

90
Q

ciculating blasts on blood smear is indicative of

A

AML

**possibly other myeloid neoplasms as well

91
Q

ciculating blasts on blood smear is indicative of

A

AML

**possibly other myeloid neoplasms as well

IBI Test 3 (20 decks)

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