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Lindsey's Blood and Lymph Unit III > Acute Leukemias > Flashcards

Acute Leukemias Flashcards

1
Q

The gene fusion in APL fuses the ______ to another, so differentiation is blocked.

A

retinoic acid receptor-alpha (RARA) gene

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2
Q

What is an Auer rod?

A

small, stick-like structures in the cytoplasm

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2
Q

What is the treatment for APL?

A

retinoic acid and arsenic salts

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3
Q

In megakaryoblastic differentiation AML, the leukemic cells may express megakaryocytic antigens ____ and ____.

A

CD41, CD61

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3
Q

AML with t(1;22) RBM15-MKL1 shows _____ and is most often seen in _____.

A

megakaryoblastic differentiation; infants with Down Syndrome

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4
Q

B-ALL with abnormalities of _____ is frequently seen in neonates and young infants.

A

11q23 MLL

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4
Q

In AML with inv(16) or t(16;16) CBFB-MYH11, there is usually a mixture of increased ____ and _____.

A

myeloblasts; monocytes

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5
Q

Chromosomal abnormalities are found in 95% of ____ and 90% of ______.

A

AML patients; ALL patients

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6
Q

______ are found in 95% of AML patients and 90% of ALL patients.

A

Chromosomal abnormalities

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6
Q

What does CEBPA indicate?

A

positive AML,NOS

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7
Q

T-ALL frequently presents with lymphoblastic lymphoma, often manifesting as a ____.

A

large mediastinal mass

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8
Q

What is therapy related AML (t-AML)?

A

AML arising 2a to DNA damage from a prior therapy, like DNA alkylating agents, topo-II inhibitors, or ionizing radiation

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9
Q

Chemotherapy, especially with _____ and ____, is a risk factor for acute leukemia.

A

DNA alkylating agents; topoisomerase-II inhibitors

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10
Q

____ more frequently occurs in adolescents and young adults.

A

T-ALL

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11
Q

What are the 5 AML chromosome translocations?

A
  1. t(8;21) RUNX1-RUNX1T1 2. inv(16) or t(16;16) CBFB-MYH11 3. t(15;17) PML-RARA APL 4. t(1;22) RBM15-MKL1 5. 11q23 MLL
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12
Q

What is Fanconi Anemia?

A

inherited aplastic anemia; auto recessive or x-linked;

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12
Q

What is the incidence of AML?

A

3/100,000

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13
Q

t-AML 2a to topoisomerase-II inhibitors usually has a latency period of ____ and has a karyotype showing _____.

A

1-2 years; rearrangement of the MLL gene

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14
Q

T-ALL frequently presents with _____, often manifesting as a large mediastinal mass.

A

lymphoblastic lymphoma

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15
Q

In adults, ALL has a remission rate of ____ and cure rates of _____.

A

60-80%; less than 50%

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15
Q

What is the prognosis in t(1;22) RBM15-MKL1? Treatment?

A

relatively good; intensive chemo

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17
Q

___% of all cases of ALL occur in children under 6yo.

A

75

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17
Q

Some cases of APL give rise to ____, which the clinician should always be watching for.

A

DIC

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18
Q

What is the average age of AML diagnosis?

A

65yo

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19
Q

What are the signs of acute anemia?

A

anemia, pallor, thrombocytopenia, hemorrhage, ecchymoses, petechiae, fever, infection, adenopathy, hepatosplenomegaly, medistinal mass

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20
Q

75% of all cases of ALL occur in _____.

A

children under 6yo

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20
Q

B-ALL with _____ accounts for 25% of childhood cases and have a very favorable prognosis.

A

t(12;21) ETV-6RUNX1

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20
Q

In _____, the leukemic cells can express monocytic antigens CD64 and CD14.

A

monocytic differentiation AML

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21
Q

Acute leukemia is ____.

A

a clonal, neoplastic proliferation of immature myeloid or lymphoid cells

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21
Q

In ____, the leukemic cells may express megakaryocytic antigens CD41 and CD61.

A

megakaryoblastic differentiation AML

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22
Q

T-ALL accounts for ____% of ALL cases.

A

25-30

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22
Q

What does CBFB do?

A

encodes the beta subunit of core binding factor (CBF)

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23
Q

ALL has a good prognosis in _____; the complete remission rate is 95% and cure rates are about 80%.

A

children

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23
Q

AML with 11q23 MLL shows some degree of _____ and has a ____ prognosis.

A

monocytic differentiation; poor

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24
Q

What are the diagnostic criteria for AML?

A

increased myeloblasts accounting for >20% of nucleated cells

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26
Q

What are the symptoms of acute anemia?

A

fatigue, malaise, dyspnea, bruising, weight loss, bone or ab pain, neuro symptoms

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26
Q

Which gene encodes for the alpha unit of core binding factor (CBF), a transcription factor needed for hematopoiesis?

A

RUNX1

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27
Q

What are the 2 major categories of acute leukemia?

A
  1. acute myeloid leukemia (AML) 2. acute lymphoblastic leukemia (ALL)
27
Q

___ frequently presents with lymphoblastic lymphoma, often manifesting as a large mediastinal mass.

A

T-ALL

27
Q

How are ALL and AML differentiated?

A

flow cytometry, immunohistochemistry, and/or morphology

28
Q

ALL has a _____ in children; the complete remission rate is 95% and cure rates are about 80%.

A

good prognosis

30
Q

T-ALL more frequently occurs in _____.

A

adolescents and young adults

31
Q

Name 5 poor prognostic factors in ALL.

A
  1. age 10 2. elevated WBC 3. slow response to therapy or residual disease after treatment 4. hypodiploidy (
33
Q

Name the 3 translocations to know in B-ALL.

A
  1. t(9;22) BCR-ABL1 2. 11q23 MLL 3. t(12;21) ETV6-RUNX1
34
Q

AML with t(8;21) RUNX1-RUNX1T1 is found in about ____% of all cases and usually is seen in _____. It usually has a ____ prognosis.

A

5; younger; relatively good

36
Q

What is Ph+ ALL?

A

the t(9;22) Philadelphia chromosome; 25% of adult B-ALL cases

37
Q

____ with abnormalities of 11q23 MLL is frequently seen in neonates and young infants.

A

B-ALL

38
Q

How can you ID a myeloblast in the microscope?

A

Auer rods

40
Q

Acute leukemia is _____ without treatment.

A

fatal

41
Q

B-ALL with t(12;21) ETV-6RUNX1 accounts for ___% of childhood cases and have a very favorable prognosis.

A

25

42
Q

AML with inv(16) or t(16;16) CBFB-MYH11 is found in about ____% of all cases and usually is seen in _____. It usually has a ____ prognosis.

A

5-10; younger; relatively good

44
Q

B-ALL accounts for ____% of all cases of ALL.

A

80-85

44
Q

The presence of ____ are the most important prognostic finding for AML,NOS.

A

molecular findings

45
Q

B-ALL with t(12;21) ETV-6RUNX1 accounts for 25% of childhood cases and have a ____ prognosis.

A

very favorable

46
Q

How are chromosomal abnormalities detected?

A

cytogenetic analysis (karyotypic and FISH), molecular analysis (RT-PCR)

48
Q

How is ALL diagnosed?

A

no set % of lymphoblasts req- WBC can be increased, decreased, or normal. Lymphoblasts have CD34 and TdT

49
Q

_______ is a clonal, neoplastic proliferation of immature myeloid or lymphoid cells.

A

Acute leukemia

50
Q

What is Bloom Syndrome?

A

auto. recessive disorder predisposing to cancer early in life; small stature, facial anomalies, immunodeficiency

51
Q

APL with t(15;17) PML-RARA has increased _____ instead of blasts.

A

abnormal promyelocytes

52
Q

What is Ataxia Telangiectasia?

A

neurogenerative disorder; abnormal eye movements, immune deficiency due to a defective chromosome 11

53
Q

The leukemic cells themselves can cause _____ or _____ problems.

A

hyperviscosity; thrombotic problems

55
Q

B-ALL with abnormalities of 11q23 MLL has a ____ prognosis.

A

poor

57
Q

B-lymphoblasts express ____, ____, and/or ____.

A

CD19, CD22, CD79a

59
Q

What has the worst prognosis of all ALLs?

A

Ph+ ALL

61
Q

What is a solid mass malignancy of precursor lymphoid cells called?

A

lymphoblasic lymphoma (LBL)

63
Q

In adults, ____ has a remission rate of 60-80% and cure rates of less than 50%.

A

ALL

64
Q

AML with inv(16) or t(16;16) CBFB-MYH11 is notable bc of the presence of ____ with _____, called _____.

A

immature eosinophils; abnormal basophilic granules; baso esos

65
Q

Why does the t(8;21) cause problems?

A

RUNX1 and CBF genes are blocked, so blood cells can’t differentiate

66
Q

APL accounts for ____% of AML, and is diagnosed when _____ is found.

A

5-10; t(15;17)

67
Q

What does FLT3 ITD indicate?

A

negative AML,NOS

69
Q

T-ALL occurs more commonly in ____ and ____.

A

males; adolescents and young adults

71
Q

What is the incidence of ALL?

A

1-5:100,000

72
Q

What antigens are only seen on immature T-cells?

A

CD99; CD1a

73
Q

T-lymphoblasts express ____, ____, ____, ____, ____, ____, and/or _____.

A

CD2, CD3, CD7, CD4, CD8, CD99, CD1a

74
Q

About 50% of all AML cases have a normal karyotype and are called ____.

A

AML-NOS (not otherwise specified)

75
Q

ALL is divided into ____ and _____.

A

B-lymphoblastic ALL (B-ALL); T-lymphoblastic ALL (T-ALL)

75
Q

The gene fusion in APL fuses the retinoic acid receptor-alpha (RARA) gene to another, so _____ is blocked.

A

differentiation

77
Q

What do the cells look like in APL?

A

promyelocytes; hypergranular; Auer rods

78
Q

What causes the s/s of acute leukemia?

A

loss of normal hematopoietic elements and blood cells

79
Q

T-ALL more frequently occurs in ____.

A

adolescents and young adults

80
Q

What is RUNX1?

A

a gene encoding for the alpha unit of core binding factor (CBF), a transcription factor needed for hematopoiesis

81
Q

B-ALL with abnormalities of 11q23 MLL is frequently seen in _____.

A

neonates and young infants

82
Q

Name 5 risk factors for acute leukemia.

A
  1. chemo (esp of DNA alkylating agents or topo inhibitors) 2. tobacco smoke 3. ionizing radiation 4. benzene exposure 5. genetic syndromes (Down, Bloom, Fanconi anemia, AT)
83
Q

What do Auer rods indicate?

A

that the cell is a myeloblast

84
Q

In ____, ALL has a remission rate of 60-80% and cure rates of less than 50%.

A

adults

86
Q

___ has a good prognosis in children; the complete remission rate is 95% and cure rates are about 80%.

A

ALL

87
Q

In monocytic differentiation AML, the leukemic cells can express monocytic antigens ____ and ____.

A

CD64, CD14

88
Q

____accounts for 10-20% of AML cases and has a very poor prognosis.

A

t-AML

89
Q

What unique antigens does a myeloblast express?

A

CD34, CD117 (C-kit), myeloperoxidase, CD33, CD13

90
Q

t-AML 2a to alkylating agents or radation usually have a latency period of ___ and has a karyotype showing ____.

A

2-8 years; whole or partial loss of chromosome 5 and/or 7

91
Q

____, _____, _____, and ______ are genetic syndromes that are risk factors for acute leukemia.

A

Down syndrome, Bloom syndrome, Fanconi anemia, ataxia telangiectasia

92
Q

The gene fusion in ____ fuses the retinoic acid receptor-alpha (RARA) gene to another, so differentiation is blocked.

A

APL

93
Q

What does NPM1 indicate?

A

positive AML, NOS

Lindsey's Blood and Lymph Unit III (10 decks)

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