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
hemorrhagic disorders of secondary hemostasis cause
soft tissue or joint bleeds
26
thrombotic disorders cause
endothelial injury stasis hypercoagulability
27
myeloid neoplasms can be caused by [3]
myeloproliferative neoplasms acute myeloid leukemia myelodysplastic syndromes
28
lymphoid neoplasms
lymphoblastic luekemia/lymphoma mature B cell neoplasms mature T cell neoplasms Hodgkin Lymphoma
29
neoplasm derived from precursor hematopoietic cells with proliferation and accumulation of blast cells
acute leukemia **proliferation without maturation
30
>20% blast increase in the bone marrow
acute leukemia
31
if untreated, rapidly progressive and fatal in weeks to months
acute
32
primarily in immature cells (blasts)
acute
33
primarily in mature or maturing cells
chronic
34
if untreated, slowly progressive and fatal in months to years
chronic
35
primary presentation in bone marrow and blood
leukemia
36
can be derived from myeloid cells or lymphoid cells
leukemia
37
primary presentation in lymph nodes or other lymphoid tissues
lymphoma
38
derived from lymphoid cells only
lymphoma
39
main types of acute leukemias [3]
acute myeloid leukemia (AML) acute lymphoblastic leukemia/lymphoma (ALL, LBL) -B-lymphoblastic -T-lymphoblastic
40
incidence increases steadily throughout adult life
AML
41
most common malignancy of childhood
ALL **85% are B-ALL, 15% T-ALL
42
age of onset demonstrates bimodal distribution, with greater incidence occurring in childhood and after 50
ALL/LBL
43
immunodeficiency states are an etiologic association for
ALL, LBL
44
alkylating agents, topoisomerase II inhibitors are etiologic associations for
AML
45
stem cell hematologic disorders are etiologic association for
AML **aplastic anemia, PNH, etc.
46
etiologic associations for AML and ALL/LBL
ionizing radiation trisomy 21 chromosomal instability syndromes
47
clinical findings associated with acute leukemias
anemia thromobocytopenia neutropenia hepatosplenomegaly
48
clinical findings specific to ALL
bone pain lymphadenopathy
49
pathologic findings of blood in acute leukemias
anemia neutropenia thrombocytopenia circulating blasts
50
pathologic findings of bone marrow in acute leukemias
increased cellularity, increased blasts
51
blast cells
52
what test determines lineage for acute leukemias?
flow cytometry
53
myeloperoxidase + TdT- CD34 CD13 CD33 CD117
AML
54
TdT+ myeloperoxidase - CD10 CD19
B-ALL
55
TdT+ myeloperoxidase - CD2 CD3 CD5 maybe CD4/8
T-ALL
56
auer rods
AML
57
auer rods
58
goal is to produce complete remission
induction therapy
59
goal is long term disease free survival
post induction therapy **i think he also called this consolidation?
60
targeted therapies are mostly
adjunctive
61
favorable markers for AML prognosis
t(15;17) -acute promyelocytic leukemia t(2;21), inv (16) **indicate loss of maturation
62
unfavorable markers for AML prognosis
>60 WBC >100,000 prior myelodysplastic syndromes therapy-related AML
63
t(15;17)
Acute promyelocytic leukemia
64
disrupts retinoic acid receptor (RAR-a) gene
Acute promyelocytic leukemia
65
abnormal promyelocyte like cells with multiple auer rods
Acute promyelocytic leukemia
66
frequently associated with DIC
Acute promyelocytic leukemia
67
ATRA used for treatment
Acute promyelocytic leukemia
68
Acute promyelocytic leukemia prognosis
favorable to intermediate
69
Acute promyelocytic leukemia
70
favorable features for ALL prognosis
precursor B-ALL 4-10 years old <10,000 per uL hyperdiploidy t(12;21)
71
unfavorable features for ALL prognosis
precursor T-ALL <2 or >10 y.o. WBC>100,00 per uL extensive organomegaly t(4;11), t(9;22)
72
response to therapy: residual disease after initial therapy
predicts outcome
73
adult ALL has a [better/worse] prognosis than childhood ALL
worse
74
t(4;11)
unfavorble for ALL
75
t(9;22)
unfavorable for ALL
76
hyperdiploidy t(12;21)
favorable for ALL
77
t(2;21)
favorable for AML
78
acute myeloid leukemia is characterized by expression of
myeloperoxidase CD34 CD13 CD33 CD117 and/or presence of auer rods
79
ALL is characterized by
T or B lymphoid markers TdT+ expression
80
the presence of ..... is diagnostic for AML
auer rods
81
hepatosplenomegaly is an unfavorable prognostic for
ALL
82
complex karyotype including the presence of 3 or more abnormalities is an unfavorable prognostic marker for
AML
83
CNS involvement with seizures is more common in
ALL
84
blasts with prominent granules are seen in
t(15;17) APL
85
the diagnosis of AML requires | [2]
blasts with myeloperoxidase expression 20% blasts in bone marrow | circulating blasts may vary
86
Auer rods are ..... but not......to diagnose AML
sufficient but not required
87
elevated lymphocytes suggest the possibility of
CLL
88
hypercellular bone marrow with <20% blasts
AML
89
what are you likely to see on blood smear of APL
shistocytes due to high correlation with DIC
90
ciculating blasts on blood smear is indicative of
AML | **possibly other myeloid neoplasms as well
91
ciculating blasts on blood smear is indicative of
AML | **possibly other myeloid neoplasms as well