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Hematology > Exam 4 > Flashcards

Exam 4 Flashcards

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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
List out the characteristics of ALL what age group tends to get it What does it stand for What determines the prognosis
Acute lymphoblastic leukemia mostly in 2-5 year olds prognosis is worse on adults
26
What are the unique signs and symptoms for B cell ALL and T cell ALL
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
27
What are the common symptoms for both B cell ALL and T cell ALL
anemia, thrombocytopenia, organomegaly like splenomegaly, hepatomegaly,
28
Describe the morphology of a blast
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
29
What are the 4 main things that a prognosis depends on
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
30
What genetic abnormality indicates a more favorable prognosis in ALL
hyperploidy- more than 46 chromosomes
31
Both B and T cells are derived from ____ progenitors
lymphoid
32
Where do CD34 present? T or B cells, or both
both
33
Where do TdT present? T or B or both
immature lymphs only B and T terminal deoxy... transferase
34
Where do HLA-DR present
on all lymphoid cells
35
What is the worst prognosis among all ALLs
Philadelphia chromosome
36
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+.
Acute leukemia T Cell ALL
37
What are the signs and symptoms of AML
acute myeloid leukemia palor, fatigue, bruising, bleeding, splenomegaly
38
What is the CBC count in AML
WBC 5-30 x 10^9/L decreased of all cells anemia, thrombocytopenia, neutropenia >20% of cells are blasts
39
What is the most notable differential finding in AML
myeloblasts in peripheral blood
40
What is tumor lysis syndrome
seen in AML metabolic complications that are caused by the breakdown of dying cancer cells causes renal failure, hyper Ca, K, Uric acid, glucose
41
Explain what this means t(8;21)(q22;q22.1); RUNX1/RUNX1T1
translocation between chromosome 8 and 21 translocation was on the q arm- long arm- Gene from chromosome has created an abnormal protein
42
What is an inversion vs translocation
translocation- one chromosome breaks off and is attached to another inversion- chromosome is rearranged, single chromosome rearranged with itself
43
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
M2 children and young adults auer rods favorable prognosis 5% of AML cases T 8 21
44
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
M4 all ages, mostly younger myeloblasts, monoblasts and promyelocytes 50% cure rate 5-8% of cases
45
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
acute promyelocytic leukemia M3 all ages, mostly young adults hypergranular pro, with auer rods no prognosis listed 5-10% of AML cases
46
What disease state is associate with APL PML-RARA, how is it diagnosed
DIC- release of primary granules causes procoagulant activity t 15:17
47
Microgranular APL what morphology help ID it What is the prognosis How rare is it
cells with no granules, butterfly or coin on coin nucleus better than any other type of AML 30-40% of APL cases
48
What treatment is used on microgranular APL
ATRA
49
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
M5 t 9:11 children monoblasts and immature monocytes- granules and vacuoles in blasts gingival and skin 6% of AML cases
50
AML with myelodysplasia how many blasts prognosis
poor prognosis at least 20% blasts
51
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)
ATRA
52
Acute myeloid leukemia not otherwise specified what is required for diagnosis
at least 20% blasts in peripheral blood
53
List out the FAB leukemias M0 M1 M2 M3 M4 M4eo M5a M5b M6 M7
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
M0 BM findings Staining
M0- Acute myeloid leukemia with minimal differenciation no evidence of cellular maturation in BM All stains are negative
55
M1 BM findings staining
Acute Myelocytic leukemia without maturation blasts are non erythroid cells <10% maturation in WBCs positive MPO or SBB stains
56
M2 age group BM findings staining
middle aged adults clear evidence of maturation, auer rods, hypercellular, MPO, SBB and CAE pos
57
M3 BM findings What translocation is it What treatment
Acute promyelocytic leukemia blasts and pros have heavy granulation, auer rod bundles DIC T 15:17- ATRA treatment
58
promyelocytes with a bund of auer rods in it, looks like a bunch of sticks
M3- APL
59
M4 BM findings Antigen testing results staining
acute myelomonocytic leukemia monocytic blasts and myeloid in peripheral and BM pos myeloid and monocytic antigens MPO, SBB and NSE pos
60
M5 BM findings staining related disease states
Acute monoblastic and monocytic leukemia most Cells are monocytic MPO NSE pos extramedullary involvement- gingival bleeding
61
M6 BM findings
80% erythroids, abnormal multinucelic, nucleus more immature than cytoplasm, vacuolization many NRBCs in peripheral blood
62
M7 BM findings staining
dry tap common, dysplastic in all cell lines, immunostaining- antigen testing
63
What is the associated with trisomy 21 and AML
down syndrome more likely to have AML
64
Practice chart on Ch 31 slide 52
practice
65
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
MPO myeloperoxidase SBB sudan black SE specific esterase NSE non specific esterases CAE chloroacetate esterase
66
What testing is used to differentiate lymphoma from leukemia
flow cytometry and immunohistochemistry
67
What are b symptoms
the physical exam, the first step in accurate diagnosis fever, drenching night sweats, loss of 10% body weight over 6 months
68
What disease is specific to alcohol intolerance, rash upon taking ampicillin
Hodgkin lymphoma
69
What disease is specific to skin issues
T cell disorders
70
What are the Ann Arbor and Lugano systems
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
What are the 5 parameters of IPI
age serum LDH performance status stage extranodal involvement
72
What leukemia is the most common in adults in western countried
CLL
73
CLL B or T cell age group diagnosis
B cell older adults asymptomatic, at least 5x 10^9 B lymphs for more than 3 months
74
What leukemia shows soccer ball cells and lots of smudged cells
lymphs- CLL
75
How is CLL treated
targeted inhibitor
76
How is the prognosis of CLL determines
with Fai and Binet staging
76
How is the prognosis of CLL determines
with Fai and Binet staging
77
PLL age group how bad is it clinical symptoms
elderly aggressive massive splenomegaly, very high lymph count skin can get infiltrated
78
Cells with cerebriform appearance
PLL- T cell
79
How to distinguish T cell PLL and B cell PLL
B cell- bigger cells, more open chromatin, more prominent nucleus T cell- smaller cells, irregular contour, cerebriform appearance
80
Treatment for PLL
Akemtuzumab- anti CD52 possible hematopoietic stem cell transplant
81
HCL age group clinical symptoms how bad is it
about 50 years splenomegaly, cytopenias indolent
82
What cancer is associated with hairy cells lymphs with round/ oval nuceli, no nucleus and ragged projections around the cell
HCL hairy cell leukemia
83
Leukemia with dry tap, and shows anti-CD20
HCL hairy cell leukemia
84
Treatment for HCL
BRAFV600E vermurafenib inhibitor for a specific mutation
85
LGL age group clinical symptoms
Large granular lymphocytis leukemia rare- older adults asymptomatic
86
Cancer where there are many pale blue cytoplasmic cells with azurophilic granules
LGL
87
Treatment for LGL
myeloid growth factors
88
What is ATLL
Adult T cell leukemia/ lymphoma T cell disorder
89
What leukemia is associated with HTLV-I
ATLL
90
What leukemia is assocaited with flower cells
ATLL
91
What is BL
Burkitt lymphoma/ leukemia B cell
92
What leukemia is associated with HIV
BL
93
What leukemia has deeply basophilic cytoplasm with vacuoles looks like a starry sky
BL
94
What is FL
Folicular lymphoma germinal B cell disorder
95
What leukemia is associated with cleft nuclei, look like hoof prints
FL
96
What is MCL
Mantle cell lymphoma
97
What leukemia is associated with GI tract issues
MCL
98
What is DLBCL
diffuse large B cell lymphoma
99
What is the most common form of NHL
DLBCL
100
What leukemia is associated with large cells with a diffuse pattern in lymph node samples
DLBCL
101
What is MZL
marginal zone lymphoma- B cells
102
What leukemia is associated with MALT
MZL
103
What cancer is associated with polar distribution or villanous lymphs
SMZL splenic MZL
104
What cancer is associated with Hep C
NMZL
105
What is MF/ SS
Mycosis fungoides/ sezary syndrome T cell lymphoma MF mostly skin SS systemic with peripheral blood- worse prognosis
106
What cancer is associated with cerebriform folded nuelus and sezary cells
MF/SS
107
Is ALCL a T cell or B cell issue
T cell- anaplastic large cell lymphoma
108
What is a plasma cell neoplasm?
disorder of differenciated B cells plasma cell secrete monoclonal immunoglobulins, becomes decreased
109
What is an M-spike
immunofixation electrophoresis, shows too many gamma globulins, spike at the end of the chart
110
What is the order of incidence of heavy chain involvement
GAMDE
111
What is associated with increase in IgG
Multiple myeloma
112
What is MM
BM plasma cell neoplasm with extension to bone or soft tissue
113
What is associated with osteolytic lesions (moth bitten X rays) and reauloux
MM
114
What treatment for MM
anti CD38 daratumumab
115
What is WM
waldenstroms macroglobulinemia lymphoplasmacytic lymphoma associated with secretion of IgM
116
What is associated with the MYD88 mutation
WM
117
What is hogdkin lymphoma
lymph node disease, rare, in young adults
118
Classic vs LPHL
classic- continuous movement through lymphs LPHL-lymphocytic predominant non contagious nodal movement- no pattern
119
How is Hodgkins lyphoma diagnosed?
lymph node biopsy
120
What is the best way to differentiate non hodgkins from hodgkins
presence of RS cells in HL absence in NHL
121
What cancer is associated with popcorn cells
Lymphocytic predominant hodkins lymphoma LPHL
122
Cancer with RS reed sternberg cells, look like owl eyes
HL

Hematology (4 decks)

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