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BIIC Cancers and Pharmo > Acute Leukemia > Flashcards

Acute Leukemia Flashcards

1
Q

White Cell Neoplasms (3)

A
  • based on origin and differentiation
    1. lymphoid neoplasms
    2. myeloid neoplasms
    3. histocytic neoplasms
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2
Q

Lymphoid Neoplasms

A

-certain leukemias
-Hodgkin
- non-Hodgkin
plasma cell dyscrasis

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3
Q

Myeloid Neoplasms

A
  • certain leukemias
  • myelodysplastic sydromes (MDS)
  • myeloproliferative neoplasms
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4
Q

Histocytic Neoplasms

A
  • Langerhans

- histiocytoses

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5
Q

Lymphoid neoplasms characteristically manifest as _________, w/ involvement of _________ and ________

A
  • leukemias

- bone marrow and peripheral blood

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6
Q

Lymphomas present in _____

A

lymph nodes or other tissues

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7
Q

Plasma cell tumors manifest as

A

discrete masses

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8
Q

All lymphoid neoplasms have the potential to spread to lymph nodes and other tissues– especially the liver, spleen, bone marrow, and peripheral blood.

A

yep

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9
Q

B and T cell tumors are composed of

A

cells arrested at or derived from a specific stage of normal lymphocyte differentiation

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10
Q

1st progenitor in normal lymphopoiesis is

A

lymphoblast

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11
Q

WHO classifies lymphoid neoplasms on (4)

A
  • morphology
  • cell of origin
  • genotype
  • clinical features
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12
Q

Acute Lymphoid Leukemias/Lymphomas/ALLsare comprised of

A

immature B(pre-B) or T(pre-T) cells aka lymyphoblasts

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13
Q

Most ALLs are_________ and manifest as childhood acute leukemias.

A

B-ALLs

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14
Q

Less common ALLs are ________ and manifest in adolescent males as thymic lymphomas.

A

T-ALLs

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15
Q

ALL is most common cause of cancer of _________

A

children

  • 2500 new cases per year–> mostly in kids under 15
  • 3x more common in whites
  • males>females
  • Hispanics have highest incidence
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16
Q

B-ALL peaks at around _____

A

age 3

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17
Q

T-ALL is in adolescence b/c this is when _______________

A

the thymus reaches max size

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18
Q

chromosomal lesions in ALL _______ the expression and fxn of __________ needed for normal differentiation of B/T cell progenitors

A
  • dysregulate

- transcription factors

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19
Q

up to 70% of T-ALLs are ________mutations in ____ that is essential in T cell differentiation

A
  • gain-of-function

- NOTCH1

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20
Q

Lots of B-ALLs have _____________ mutations in genes required for B cell differentiation.

A
  • loss-of-function

- Eg. PAX5

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21
Q

Varied mutations in T-/B-ALLs __________________

A

promote maturation arrest and increased self-renewal –> seen in immortalized cells

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22
Q

Mutations in genes are NOT sufficient to produce ALL

A

preach

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23
Q

Lesions that drive cell growth –> mutations that increase tyrosine kinase activity and RAS signaling are what?

A

also seen in ALL

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24
Q

what does a bone marrow look like in ALL?

A

hypercellular and packed with lymphoblasts

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25
Q

Mediastinal masses occur in 50-70% in _______ which are more likely to be associated with ___________`

A
  • T-ALLs

- lymphdenopathy and splenomegaly

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26
Q

ALL is _______ (another word for really bad)

A

aggressive

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27
Q

What is one reason ALL is so aggressive?

A

high mitotic rate

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28
Q

B-/T-ALLs –> histo

A
  • scant basophilic cytoplasm
  • nuclei w/ delicate/fine;y stipple chromatin
  • small nuclei
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29
Q

Appearance of blasts is identical in Pre-B/Pre-T ALLs

A

can’t rule out other AML just based on this

-DX RELIES ON IMMUNOPHENOGENIC STUDIES

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30
Q

Peripheral blood findings are ___________

A

highly variable

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31
Q

WBC can be ____________ or __________

A

high = over 100,000 OR low = under 10,000

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32
Q

some pt with ALL might have no circulating blasts =

A

aleukemic leukemia

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33
Q

ALL - 3 common peripheral blood findings

A
  • anemia
  • low platelet count below 100,000
  • neutropenia
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34
Q

_____% of ALLs have ______________abnormalities

A
  • 90

- karyotypic

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35
Q

Childhood pre-B cell tumors are ______ AND have a _______ translocation involving the ETV6 and RUNX1 genes

A
  • hyperdiploidy
  • (12:21)
  • creates a fusion gene encoding an aberrant transcription factor
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36
Q

ABL and BCR translocation

A

t(9:22) – in 25% of adult pre-C cell tumors

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37
Q

Pre-T cell tumors have __________

A

diverse chromosomal changes

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38
Q

Terminal deoxynucleotidyl transferase (TdT)

A

enzyme specifically expressed in pre-B and pre-T cells –> present in more than 95% cases

39
Q

ALL B-cell markers

A

TdT+ and CD19+

40
Q

ALL T-cell markers

A

TdT+ and CD2

41
Q

Most pts present w/n a few weeks of the onset of symptoms

A

yep

42
Q

ALL Clinical Features: Related to Depression of Marrow Function (4)

A
  • fatigue resulting from anemia
  • fever
  • reflecting infections secondary to neutropenia
  • bleeding due to thrombocytopenia
43
Q

ALL Clinical Features: Caused by Neoplastic Infiltration (4)

A
  • generalized lymphadenopathy
  • splenomegaly
  • hepatomegaly
  • complications related to compression of large vessels and areas in mediastinum (T-ALL only)
44
Q

ALL Clinical Features: CNS Manifestations (4)

A
  • result from meningeal spread
  • headache
  • vomiting
  • nerve palsies
45
Q

In children, what is the outlook? (ALL)

A

95% obtain remission and 75-85% are cured

46
Q

ALL is the ____________________ in children.

A

the leading cause of cancer deaths

47
Q

In adults, what is the outlook? (ALL)

A

35-40% cured

48
Q

4 Factors associated with WORSE prognosis (ALL)

A
  • younger than 2 yr
  • adolescence/adulthood
  • peripheral blood blast counts greater than 100,000
  • detection of residual disease AFTER therapy
49
Q

3 Factors associated with a FAVORABLE prognosis (ALL)

A
  • 2-10yr
  • low white cell count
  • hyperdiploidy
50
Q

Treatment of BCR-ABL (9;22)

A

BCR-ABL kinase inhibitors in combo with chemo

51
Q

Why is the outlook for adults with ALL guarded?

A

adults cannot tolerate the intensive chemo that is curative in kids

52
Q

Key Concept: ALL are highly aggressive tumors that manifest with signs and symptoms of ____________________ or as ___________

A
  • bone marrow failure

- rapidly growing masses

53
Q

Key Concept: In ALL, tumor cells contain genetic lesions that ______________, leading to the accumulation of _______________

A
  • block differentiation

- immature, nonfunctional blasts

54
Q

Granulopoiesis

A

synthesis of azurophilic granules and specific granules in the cytoplasm

55
Q

Myeloblast

A

most immature of the myeloid series

56
Q

Promyelocyte

A

basophilic cytoplasm and azurophilic granules containing lysosomal enzymes

57
Q

Myelocyte

A

specific granules appear

58
Q

Metamyelocyte

A

increasing number of specific granules

59
Q

Band Cell

A

nucleus but not yet polymorphic

60
Q

Segmented granulocytes

A

maturation

61
Q

Myeloid Neoplasms arise from _____________ and give rise to _____________

A
  • hematopoietic progenitors

- proliferation that involve the bone marrow and replace normal marrow elements

62
Q

Myeloid Neoplasia: Acute Myeloid Leukemia (AML)

A
  • neoplastic cells are BLOCKED at an early stage

- immature myeloblasts accumulate in marrow, replace normal elements, and circulate peripheral blood

63
Q

Myeloid Neoplasia: Myelodysplastic Syndrome (MDS)

A

-terminal differentiation occurs in a DISORDERED and INEFFECTIVE way —> dysplastic marrow precursors and peripheral blood cytopenias

64
Q

Myeloid Neoplasia: Myeloproliferative Neoplasms

A
  • neoplastic clones continues to undergo terminal differentiation but exhibits INCREASED OR DYSREGULATED GROWTH
  • increase in one or more of the formed elements in the peripheral blood
65
Q

AML primarily affects_______ and the median age is

A
  • older adults

- 50 years

66
Q

AML symptoms are usually related to __________________

A

the replacement of normal marrow elements by leukemic blasts

67
Q

AML: symptoms (5) - present w/n a few weeks of onset of symptoms

A
  • fatigue
  • pallor
  • abnormal bleeding
  • infections
  • granulocytic sarcoma (manifesting in discrete tissue mass)
68
Q

AMLs harbor mutations ___________

A

in transcription factors
- interfere with the differentiation of early myeloid cells—> leading to the accumulation of myueloid precursors in the marrow

69
Q

AML subset: Acute Promyelocytic Leukemia (APL): Mutation

A

t(15;17)
-fusion of the retinoic acid receptor alpha (RARA) gene on chromosome 17 and the PML gene on chromosome 15 = PML/RARA fusion protein blocks myeloid differentiation at the promyelocytic stage

70
Q

AML subset: APL: Treatment

A

all-trans retinoic acid (ATRA) = analogue of vitamin of A—> induces the neoplastic promyelocytes to differentiate into neutrophils
-rapid clearance of tumor

71
Q

AML, what does the bone marrow look like?

A

AML myeloid blasts or promyelocytes make up more than 20% of the marrow

72
Q

Auer Rods

A

red staining rod-like structures in myeloblasts or more differentiated cells
–MORE numerous in APL

73
Q

In some subtypes of AML _______, _____________, and ____________ predominate

A
  • monoblasts
  • erythroblasts
  • megakaryocytes
74
Q

AMLs are DIVERSE in terms of … (3)

A
  • genetics
  • cellular lineage
  • degree of mutation
75
Q

WHO classification of AML (4)

A
  • specific genetic aberrations
  • dysplasia
  • occurring after genotoxic chemo
  • lacking any of the above–> these are further subclassified
76
Q

AML: FAB Classification also exists honestly idk if we had to know if but like its just progressively worse from M0 to M7

A

eh

77
Q

AML: Immunophenotype

A

CD13, CD14, CD15, CD33, CD64, CD117(KIT)

- CD34 present on myeloblasts

78
Q

AML: Clinical Features: Symptoms (6)

A
  • anemia
  • neutropenia
  • thrombocytopenia
  • fatigue
  • fever
  • spontaneous mucosal and cutaneous bleeding
79
Q

AML: Clinical Features: Thrombocytopenia

A
  • results in bleeding disorder w/ cutaneous petechiae and eccymoses, serosal hemorrhages, and mucosal hemorrhages
  • procoagulants and fibrinolytic factors released by leukemic cells EXACERBATE BLEEDING TENDNCY—> DIC
80
Q

AML: Clinical Features: Infections

A
  • frequent

- caused by OPPORTUNISTS

81
Q

AML: Clinical Features: CNS

A

CNS less common than ALL

82
Q

AML: Clinical Features: Tumors with “Good-Risk” Karyotypic Abnormalitites

A
  • t(8;21) and inv(16)

- 50% long-term disease-free survival but OVERALL survival in all pt is 15-30%

83
Q

AML: Clinical Features: TP53 Mutations

A

particularly poor prognosis

84
Q

Myelodysplastic Syndrome (MDS)

A

clonal stem cell disorders characterized by maturation defects associated with INEFFECTIVE hematopoiesis and HIGH RISK OF TRANSFORMATION to AML

85
Q

MDS: bone marrow

A

-hypercellular and normocellular
partly or wholly replaced by clonal progeny of transformed multipotent stem cell that retains the capacity to differentiate into RBCs, granulocytes, and platelets but in a manner that is both INEFFECTIVE AND DISORDERED

86
Q

MDS: peripheral blood

A

-one or more cytopenias

87
Q

MDS: stem cell clone

A

genetically unstable and prone to acquisition of additional mutations and the eventual transformation to AML
-most cases are IDIOPATHIC

88
Q

MDS: Mutated Gene Categories (3)

A
  • epigenetic factors
  • RNA splicing factors
  • transcription factors
  • some have loss-of-function mutations in TP53=tumor suppressor gene–> these correlate with complex karyotype and poor prognosis
89
Q

MDS: Recurrent Chromosomal Abnormalities

A
  • monosomies 5 and 7
  • deletions of 5q, 7q, and 20q
  • trisomy 8
90
Q

MDS: bone marrow: abnormal-appearing hematopoietic precursors

A
  • megaloblastoid erythroid precursors
  • ring sideroblasts(iron deposits in mitochondria of erythroid forms)
  • granulocyte precursors with abnormal granules or nuclear mutations
  • small megakaryocytes
91
Q

MDS: Clinical Features: How Common?

A
  • affects 15,000 pts per year

- age between 50-70yr

92
Q

MDS: Clinical Features: Symptoms

A
  • as a result of cytopenias—> infections
  • related to anemia
  • hemorrhages
93
Q

MDS: Clinical Features: Prognosis

A
  • conventional chemo = poor
  • transformation to AML = 10-40% of pts
  • median survival time = 9-29mos
    - worse in pts w/ increased marrow blasts, cytogenic abnormalities, or TP53 mutations
94
Q

Common mutations in Myeloproliferative Disorders

A
  • BCR-ABL
  • mutated JAK2
  • lead to constitutive activation of tyrosine kinases which mimic signals from normal growth factors

BIIC Cancers and Pharmo (10 decks)

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