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Hematology > Exam 4 > Flashcards

Exam 4 Flashcards

1
Q

What kind of genetic issue is there with the Philadelphia chromosome

A

translocation on chromosome 9 and 22 (t9,22)

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2
Q

What is the difference between a leukemia and a lymphoma

A

leukemia- originate in the BM, cells get into peripheral blood, lymphoid tissues and other organs
Lymphoma- originate in the lymphatic system, cells can circulate in peripheral blood

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3
Q

Describe the differences between acute and chronic leukemias

A

acute- sudden, onset, rapid, quicker death, variable counts, excess blasts
chronic- insidious/ slow, longer survival, usually high WBC count, too many mature cells

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4
Q

What are the 3 hematologic neoplasms

A

leukemias
lymphomas and
myelodysplastic syndromes (MDS)

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5
Q

What leukemia is more common in children

A

ALL- Acute lymphoblastic leukemia

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6
Q

What leukemia is more common in adults

A

CLL- Chronic lymphocytic leukemia

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7
Q

Describe FAB vs WHO classifications of leukemias

A

FAB- based on morphology
WGO- more precise, based on clinical features, genetics, morphology

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8
Q

What are epigenetic mechanisms

A

mechanics that control how genes are expressed and silenced

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9
Q

What are protooncogenes

A

encode for proteins that are essential for normal cell function.

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10
Q

What is an oncogene

A

a mutation of a protooncogene with leukemogenic potential
if activated could become malignant

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11
Q

What is a qualitative mutation

A

structural change to the protooncogene and production of an abnormal protein product

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12
Q

What is a quantitative mutation

A

overexpression of a normal protooncogene in a HSC

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13
Q

What are tumor suppressor genes

A

proteins that protect cells from malignant transformation

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14
Q

How can tumor suppressor genes promote malignant transformation

A

if they are inactivated or deleted

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15
Q

Which is dominant and which is recessesive
tumor suppressor genes and oncogenes

A

tumor suppressor- recessive
oncogene- dominant

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16
Q

What are the 2 classification of chemotherapies

A

Phase specific- affects cell cycle only in specific phases, or phase non specific
Mechanism of action- what agent is used to kill cancer cells, alkylating agents … etc

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17
Q

What are the 3 types of remission

A

hematologic- normal BM, blood cells are recovered, no evidence of leukemic cells
cytogenetic- karyotyping used to determine no leukemic cells
molecular- no leukemic cell nucleic acids are present

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18
Q

What is targeted therapy

A

act on specific malignant cells leaving normal ones untouched.

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19
Q

What is Gleevac

A

a molecular targeted therapy for chronic phase CML
tyrosine kinase inhibitor induces apoptosis of CML cells

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20
Q

What is ATRA

A

all trans retinoic acid
treatment for pts with APL with PML RARA gene

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21
Q

What is Rituximab

A

monoclonal ab treatment for lymphoid neoplasms
anti-CD20

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22
Q

What are the 3 types of HSC donors

A

syngeneic- from an identical twin
allogeneic-from an HLA-identical sibling or unrelated donor
autologous- donating to yourself

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23
Q

What are the WHO and FAB requirements to consider a diagnosis of acute leukemias

A

FAB- 30% blasts in BM or peripheral blood
WHO- 20% blasts in BM or peripheral blood

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24
Q

A pt with 21% blasts in their peripheral blood. Do they have acute leukemia based on the FAB an WHO standards?

A

FAB- not acute leukemia
WHO- acute leukemia

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25
Q

List out the characteristics of ALL
what age group tends to get it
What does it stand for
What determines the prognosis

A

Acute lymphoblastic leukemia
mostly in 2-5 year olds
prognosis is worse on adults

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26
Q

What are the unique signs and symptoms for B cell ALL and T cell ALL

A

B cell- bone pain caused by intramedullary growth of leukemic cells, can infiltrate meninges
T cell- mass in mediastinum/ chest lump in X ray, also bone pain

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27
Q

What are the common symptoms for both B cell ALL and T cell ALL

A

anemia, thrombocytopenia, organomegaly like splenomegaly, hepatomegaly,

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28
Q

Describe the morphology of a blast

A

1-2.5 x the size of a normal lymph
scant blue cytoplasm
indistinct nucleoli
or could be large- 2-3x normal
prominent nucleoli
membrane irregularities

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29
Q

What are the 4 main things that a prognosis depends on

A

lymphoblast load- tumor burden
immunophenotype- flow cytometry results
age- children especially toddlers have better prognosis
genetic abnormalities- chromosomal translocations depending on which kind can have good or bad prognosis

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30
Q

What genetic abnormality indicates a more favorable prognosis in ALL

A

hyperploidy- more than 46 chromosomes

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31
Q

Both B and T cells are derived from ____ progenitors

A

lymphoid

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32
Q

Where do CD34 present? T or B cells, or both

A

both

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33
Q

Where do TdT present? T or B or both

A

immature lymphs only B and T
terminal deoxy… transferase

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34
Q

Where do HLA-DR present

A

on all lymphoid cells

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35
Q

What is the worst prognosis among all ALLs

A

Philadelphia chromosome

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36
Q

12-year-old presents with complaints of easy bruising and fatigue. X-ray shows presence of a mediastinal mass. BM biopsy shows 45% blasts. Immunophenotyping shows blasts are CD2+, CD3+, CD4+, CD8+, CD34+, and TdT+.

A

Acute leukemia T Cell ALL

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37
Q

What are the signs and symptoms of AML

A

acute myeloid leukemia
palor, fatigue, bruising, bleeding, splenomegaly

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38
Q

What is the CBC count in AML

A

WBC 5-30 x 10^9/L
decreased of all cells
anemia, thrombocytopenia, neutropenia
>20% of cells are blasts

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39
Q

What is the most notable differential finding in AML

A

myeloblasts in peripheral blood

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40
Q

What is tumor lysis syndrome

A

seen in AML
metabolic complications that are caused by the breakdown of dying cancer cells
causes renal failure, hyper Ca, K, Uric acid, glucose

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41
Q

Explain what this means
t(8;21)(q22;q22.1); RUNX1/RUNX1T1

A

translocation between chromosome 8 and 21
translocation was on the q arm- long arm-
Gene from chromosome has created an abnormal protein

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42
Q

What is an inversion vs translocation

A

translocation- one chromosome breaks off and is attached to another
inversion- chromosome is rearranged, single chromosome rearranged with itself

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43
Q

AML with
Put into FAB nomenclature
What age group tends to get it
what morphology help ID it
What is the prognosis
How rare is it

A

M2
children and young adults
auer rods
favorable prognosis
5% of AML cases
T 8 21

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44
Q

AML with INV 16 or T 16:16
Put into FAB nomenclature
What age group tends to get it
what morphology help ID it
What is the prognosis
How rare is it

A

M4
all ages, mostly younger
myeloblasts, monoblasts and promyelocytes
50% cure rate
5-8% of cases

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45
Q

APL with PML RARA t 15:17
Put into FAB nomenclature
What age group tends to get it
what morphology help ID it
What is the prognosis
How rare is it

A

acute promyelocytic leukemia M3
all ages, mostly young adults
hypergranular pro, with auer rods
no prognosis listed
5-10% of AML cases

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46
Q

What disease state is associate with APL PML-RARA, how is it diagnosed

A

DIC- release of primary granules causes procoagulant activity
t 15:17

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47
Q

Microgranular APL
what morphology help ID it
What is the prognosis
How rare is it

A

cells with no granules, butterfly or coin on coin nucleus
better than any other type of AML
30-40% of APL cases

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48
Q

What treatment is used on microgranular APL

A

ATRA

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49
Q

AML
Put into FAB nomenclature
What age group tends to get it
what morphology help ID it
What disease is it associated with
How rare is it

A

M5 t 9:11
children
monoblasts and immature monocytes- granules and vacuoles in blasts
gingival and skin
6% of AML cases

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50
Q

AML with myelodysplasia
how many blasts
prognosis

A

poor prognosis
at least 20% blasts

51
Q

Patient is experiencing DIC
Differential shows 34% blasts in peripheral blood
Many Auer rods- only in myeloid
Cytogenetic testing shows presence of t(15;17)

A

ATRA

52
Q

Acute myeloid leukemia not otherwise specified
what is required for diagnosis

A

at least 20% blasts in peripheral blood

53
Q

List out the FAB leukemias
M0
M1
M2
M3
M4
M4eo
M5a
M5b
M6
M7

A

M0- acute myeloid leukemia, minimally differentiated
M1-acute myeloid leukemia, without maturation
M2- Acute myeloid leukemia with maturation
M3 -Acute promyeloid leukemia
M4- acute myelomonocytic leukemia
M4eo Acute myelomonocytic leukemia with eosinophilia
M5a- Acute monocytic leukemia, poorly differenciated
M5b- Acute monocytic leukemia, well differenciated
M6- Acute erythroleukemia
M7 Acute megakaryocytic Leukemia

54
Q

M0
BM findings
Staining

A

M0- Acute myeloid leukemia with minimal differenciation
no evidence of cellular maturation in BM
All stains are negative

55
Q

M1
BM findings
staining

A

Acute Myelocytic leukemia without maturation
blasts are non erythroid cells <10% maturation in WBCs
positive MPO or SBB stains

56
Q

M2
age group
BM findings
staining

A

middle aged adults
clear evidence of maturation, auer rods, hypercellular,
MPO, SBB and CAE pos

57
Q

M3
BM findings
What translocation is it
What treatment

A

Acute promyelocytic leukemia
blasts and pros have heavy granulation, auer rod bundles
DIC
T 15:17- ATRA treatment

58
Q

promyelocytes with a bund of auer rods in it, looks like a bunch of sticks

A

M3- APL

59
Q

M4
BM findings
Antigen testing results
staining

A

acute myelomonocytic leukemia
monocytic blasts and myeloid in peripheral and BM
pos myeloid and monocytic antigens
MPO, SBB and NSE pos

60
Q

M5
BM findings
staining
related disease states

A

Acute monoblastic and monocytic leukemia
most Cells are monocytic
MPO NSE pos
extramedullary involvement- gingival bleeding

61
Q

M6
BM findings

A

80% erythroids, abnormal multinucelic, nucleus more immature than cytoplasm, vacuolization
many NRBCs in peripheral blood

62
Q

M7
BM findings
staining

A

dry tap common, dysplastic in all cell lines,
immunostaining- antigen testing

63
Q

What is the associated with trisomy 21 and AML

A

down syndrome more likely to have AML

64
Q

Practice chart on Ch 31 slide 52

A

practice

65
Q

Match to stain
-enzyme in primary granules of granulocytic cells, does not stain lymphs
-stains the cellular lipids, staining is more intense as cells mature, does not stain lymphs
-only stains granulocytes, stains esters
-stains esters in all cells not just granulocytes
-stains primary granules in neutrophils

A

MPO myeloperoxidase
SBB sudan black
SE specific esterase
NSE non specific esterases
CAE chloroacetate esterase

66
Q

What testing is used to differentiate lymphoma from leukemia

A

flow cytometry and immunohistochemistry

67
Q

What are b symptoms

A

the physical exam, the first step in accurate diagnosis
fever, drenching night sweats, loss of 10% body weight over 6 months

68
Q

What disease is specific to alcohol intolerance, rash upon taking ampicillin

A

Hodgkin lymphoma

69
Q

What disease is specific to skin issues

A

T cell disorders

70
Q

What are the Ann Arbor and Lugano systems

A

Ann Arbor- classifies lymphomas by presence or absence of B symptoms, nodes above diaphragm, stages 1 and 2, nodes on both halves- stages 3 or 4
Lugano- classifies into stages I and II- limited, III and IV- advanced

71
Q

What are the 5 parameters of IPI

A

age
serum LDH
performance status
stage
extranodal involvement

72
Q

What leukemia is the most common in adults in western countried

A

CLL

73
Q

CLL
B or T cell
age group
diagnosis

A

B cell
older adults
asymptomatic, at least 5x 10^9 B lymphs for more than 3 months

74
Q

What leukemia shows soccer ball cells and lots of smudged cells

A

lymphs- CLL

75
Q

How is CLL treated

A

targeted inhibitor

76
Q

How is the prognosis of CLL determines

A

with Fai and Binet staging

76
Q

How is the prognosis of CLL determines

A

with Fai and Binet staging

77
Q

PLL
age group
how bad is it
clinical symptoms

A

elderly
aggressive
massive splenomegaly, very high lymph count
skin can get infiltrated

78
Q

Cells with cerebriform appearance

A

PLL- T cell

79
Q

How to distinguish T cell PLL and B cell PLL

A

B cell- bigger cells, more open chromatin, more prominent nucleus
T cell- smaller cells, irregular contour, cerebriform appearance

80
Q

Treatment for PLL

A

Akemtuzumab- anti CD52
possible hematopoietic stem cell transplant

81
Q

HCL
age group
clinical symptoms
how bad is it

A

about 50 years
splenomegaly, cytopenias
indolent

82
Q

What cancer is associated with hairy cells lymphs with round/ oval nuceli, no nucleus and ragged projections around the cell

A

HCL hairy cell leukemia

83
Q

Leukemia with dry tap, and shows anti-CD20

A

HCL hairy cell leukemia

84
Q

Treatment for HCL

A

BRAFV600E vermurafenib
inhibitor for a specific mutation

85
Q

LGL
age group
clinical symptoms

A

Large granular lymphocytis leukemia
rare- older adults
asymptomatic

86
Q

Cancer where there are many pale blue cytoplasmic cells with azurophilic granules

A

LGL

87
Q

Treatment for LGL

A

myeloid growth factors

88
Q

What is ATLL

A

Adult T cell leukemia/ lymphoma
T cell disorder

89
Q

What leukemia is associated with HTLV-I

A

ATLL

90
Q

What leukemia is assocaited with flower cells

A

ATLL

91
Q

What is BL

A

Burkitt lymphoma/ leukemia
B cell

92
Q

What leukemia is associated with HIV

A

BL

93
Q

What leukemia has deeply basophilic cytoplasm with vacuoles looks like a starry sky

A

BL

94
Q

What is FL

A

Folicular lymphoma germinal B cell disorder

95
Q

What leukemia is associated with cleft nuclei, look like hoof prints

A

FL

96
Q

What is MCL

A

Mantle cell lymphoma

97
Q

What leukemia is associated with GI tract issues

A

MCL

98
Q

What is DLBCL

A

diffuse large B cell lymphoma

99
Q

What is the most common form of NHL

A

DLBCL

100
Q

What leukemia is associated with large cells with a diffuse pattern in lymph node samples

A

DLBCL

101
Q

What is MZL

A

marginal zone lymphoma- B cells

102
Q

What leukemia is associated with MALT

A

MZL

103
Q

What cancer is associated with polar distribution or villanous lymphs

A

SMZL splenic MZL

104
Q

What cancer is associated with Hep C

A

NMZL

105
Q

What is MF/ SS

A

Mycosis fungoides/ sezary syndrome
T cell lymphoma
MF mostly skin
SS systemic with peripheral blood- worse prognosis

106
Q

What cancer is associated with cerebriform folded nuelus and sezary cells

A

MF/SS

107
Q

Is ALCL a T cell or B cell issue

A

T cell- anaplastic large cell lymphoma

108
Q

What is a plasma cell neoplasm?

A

disorder of differenciated B cells
plasma cell secrete monoclonal immunoglobulins, becomes decreased

109
Q

What is an M-spike

A

immunofixation electrophoresis, shows too many gamma globulins, spike at the end of the chart

110
Q

What is the order of incidence of heavy chain involvement

A

GAMDE

111
Q

What is associated with increase in IgG

A

Multiple myeloma

112
Q

What is MM

A

BM plasma cell neoplasm with extension to bone or soft tissue

113
Q

What is associated with osteolytic lesions (moth bitten X rays) and reauloux

A

MM

114
Q

What treatment for MM

A

anti CD38 daratumumab

115
Q

What is WM

A

waldenstroms macroglobulinemia
lymphoplasmacytic lymphoma
associated with secretion of IgM

116
Q

What is associated with the MYD88 mutation

A

WM

117
Q

What is hogdkin lymphoma

A

lymph node disease, rare, in young adults

118
Q

Classic vs LPHL

A

classic- continuous movement through lymphs
LPHL-lymphocytic predominant non contagious nodal movement- no pattern

119
Q

How is Hodgkins lyphoma diagnosed?

A

lymph node biopsy

120
Q

What is the best way to differentiate non hodgkins from hodgkins

A

presence of RS cells in HL
absence in NHL

121
Q

What cancer is associated with popcorn cells

A

Lymphocytic predominant hodkins lymphoma
LPHL

122
Q

Cancer with RS reed sternberg cells, look like owl eyes

A

HL

Hematology (4 decks)

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