Acute Leukemias Flashcards

(93 cards)

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
What are the signs of acute anemia?
anemia, pallor, thrombocytopenia, hemorrhage, ecchymoses, petechiae, fever, infection, adenopathy, hepatosplenomegaly, medistinal mass
20
75% of all cases of ALL occur in \_\_\_\_\_.
children under 6yo
20
B-ALL with _____ accounts for 25% of childhood cases and have a very favorable prognosis.
t(12;21) ETV-6RUNX1
20
In \_\_\_\_\_, the leukemic cells can express monocytic antigens CD64 and CD14.
monocytic differentiation AML
21
Acute leukemia is \_\_\_\_.
a clonal, neoplastic proliferation of immature myeloid or lymphoid cells
21
In \_\_\_\_, the leukemic cells may express megakaryocytic antigens CD41 and CD61.
megakaryoblastic differentiation AML
22
T-ALL accounts for \_\_\_\_% of ALL cases.
25-30
22
What does CBFB do?
encodes the beta subunit of core binding factor (CBF)
23
ALL has a good prognosis in \_\_\_\_\_; the complete remission rate is 95% and cure rates are about 80%.
children
23
AML with 11q23 MLL shows some degree of _____ and has a ____ prognosis.
monocytic differentiation; poor
24
What are the diagnostic criteria for AML?
increased myeloblasts accounting for \>20% of nucleated cells
26
What are the symptoms of acute anemia?
fatigue, malaise, dyspnea, bruising, weight loss, bone or ab pain, neuro symptoms
26
Which gene encodes for the alpha unit of core binding factor (CBF), a transcription factor needed for hematopoiesis?
RUNX1
27
What are the 2 major categories of acute leukemia?
1. acute myeloid leukemia (AML) 2. acute lymphoblastic leukemia (ALL)
27
\_\_\_ frequently presents with lymphoblastic lymphoma, often manifesting as a large mediastinal mass.
T-ALL
27
How are ALL and AML differentiated?
flow cytometry, immunohistochemistry, and/or morphology
28
ALL has a _____ in children; the complete remission rate is 95% and cure rates are about 80%.
good prognosis
30
T-ALL more frequently occurs in \_\_\_\_\_.
adolescents and young adults
31
Name 5 poor prognostic factors in ALL.
1. age 10 2. elevated WBC 3. slow response to therapy or residual disease after treatment 4. hypodiploidy (
33
Name the 3 translocations to know in B-ALL.
1. t(9;22) BCR-ABL1 2. 11q23 MLL 3. t(12;21) ETV6-RUNX1
34
AML with t(8;21) RUNX1-RUNX1T1 is found in about \_\_\_\_% of all cases and usually is seen in \_\_\_\_\_. It usually has a ____ prognosis.
5; younger; relatively good
36
What is Ph+ ALL?
the t(9;22) Philadelphia chromosome; 25% of adult B-ALL cases
37
\_\_\_\_ with abnormalities of 11q23 MLL is frequently seen in neonates and young infants.
B-ALL
38
How can you ID a myeloblast in the microscope?
Auer rods
40
Acute leukemia is _____ without treatment.
fatal
41
B-ALL with t(12;21) ETV-6RUNX1 accounts for \_\_\_% of childhood cases and have a very favorable prognosis.
25
42
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.
5-10; younger; relatively good
44
B-ALL accounts for \_\_\_\_% of all cases of ALL.
80-85
44
The presence of ____ are the most important prognostic finding for AML,NOS.
molecular findings
45
B-ALL with t(12;21) ETV-6RUNX1 accounts for 25% of childhood cases and have a ____ prognosis.
very favorable
46
How are chromosomal abnormalities detected?
cytogenetic analysis (karyotypic and FISH), molecular analysis (RT-PCR)
48
How is ALL diagnosed?
no set % of lymphoblasts req- WBC can be increased, decreased, or normal. Lymphoblasts have CD34 and TdT
49
\_\_\_\_\_\_\_ is a clonal, neoplastic proliferation of immature myeloid or lymphoid cells.
Acute leukemia
50
What is Bloom Syndrome?
auto. recessive disorder predisposing to cancer early in life; small stature, facial anomalies, immunodeficiency
51
APL with t(15;17) PML-RARA has increased _____ instead of blasts.
abnormal promyelocytes
52
What is Ataxia Telangiectasia?
neurogenerative disorder; abnormal eye movements, immune deficiency due to a defective chromosome 11
53
The leukemic cells themselves can cause _____ or _____ problems.
hyperviscosity; thrombotic problems
55
B-ALL with abnormalities of 11q23 MLL has a ____ prognosis.
poor
57
B-lymphoblasts express \_\_\_\_, \_\_\_\_, and/or \_\_\_\_.
CD19, CD22, CD79a
59
What has the worst prognosis of all ALLs?
Ph+ ALL
61
What is a solid mass malignancy of precursor lymphoid cells called?
lymphoblasic lymphoma (LBL)
63
In adults, ____ has a remission rate of 60-80% and cure rates of less than 50%.
ALL
64
AML with inv(16) or t(16;16) CBFB-MYH11 is notable bc of the presence of ____ with \_\_\_\_\_, called \_\_\_\_\_.
immature eosinophils; abnormal basophilic granules; baso esos
65
Why does the t(8;21) cause problems?
RUNX1 and CBF genes are blocked, so blood cells can't differentiate
66
APL accounts for \_\_\_\_% of AML, and is diagnosed when _____ is found.
5-10; t(15;17)
67
What does FLT3 ITD indicate?
negative AML,NOS
69
T-ALL occurs more commonly in ____ and \_\_\_\_.
males; adolescents and young adults
71
What is the incidence of ALL?
1-5:100,000
72
What antigens are only seen on immature T-cells?
CD99; CD1a
73
T-lymphoblasts express \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, and/or \_\_\_\_\_.
CD2, CD3, CD7, CD4, CD8, CD99, CD1a
74
About 50% of all AML cases have a normal karyotype and are called \_\_\_\_.
AML-NOS (not otherwise specified)
75
ALL is divided into ____ and \_\_\_\_\_.
B-lymphoblastic ALL (B-ALL); T-lymphoblastic ALL (T-ALL)
75
The gene fusion in APL fuses the retinoic acid receptor-alpha (RARA) gene to another, so _____ is blocked.
differentiation
77
What do the cells look like in APL?
promyelocytes; hypergranular; Auer rods
78
What causes the s/s of acute leukemia?
loss of normal hematopoietic elements and blood cells
79
T-ALL more frequently occurs in \_\_\_\_.
adolescents and young adults
80
What is RUNX1?
a gene encoding for the alpha unit of core binding factor (CBF), a transcription factor needed for hematopoiesis
81
B-ALL with abnormalities of 11q23 MLL is frequently seen in \_\_\_\_\_.
neonates and young infants
82
Name 5 risk factors for acute leukemia.
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
What do Auer rods indicate?
that the cell is a myeloblast
84
In \_\_\_\_, ALL has a remission rate of 60-80% and cure rates of less than 50%.
adults
86
\_\_\_ has a good prognosis in children; the complete remission rate is 95% and cure rates are about 80%.
ALL
87
In monocytic differentiation AML, the leukemic cells can express monocytic antigens ____ and \_\_\_\_.
CD64, CD14
88
\_\_\_\_accounts for 10-20% of AML cases and has a very poor prognosis.
t-AML
89
What unique antigens does a myeloblast express?
CD34, CD117 (C-kit), myeloperoxidase, CD33, CD13
90
t-AML 2a to alkylating agents or radation usually have a latency period of ___ and has a karyotype showing \_\_\_\_.
2-8 years; whole or partial loss of chromosome 5 and/or 7
91
\_\_\_\_, \_\_\_\_\_, \_\_\_\_\_, and ______ are genetic syndromes that are risk factors for acute leukemia.
Down syndrome, Bloom syndrome, Fanconi anemia, ataxia telangiectasia
92
The gene fusion in ____ fuses the retinoic acid receptor-alpha (RARA) gene to another, so differentiation is blocked.
APL
93
What does NPM1 indicate?
positive AML, NOS