Precursor B and T cells neoplasms/ mature B and T 2 Flashcards

1
Q

Most ALL are what type of cells

A

pre-B cells

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2
Q

pre-B ALL and pre-T ALL which ones are usually leukemic or lymphomic

A

B: leukemic
T: lymphomic

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3
Q

what is the common cancer of Children

A

ALL

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4
Q

at what age is Pre-B ALL have the highest incidence

A

4

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5
Q

at what age is Pre-T ALL have the highest incidence

A

adolescence

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6
Q

when do the symptoms appear for precursor B- and T- cell neoplasms

A

“stormy” onset

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7
Q

If a child says they have bone pain, what should be in your differential diagnosis

A

ALL

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8
Q

Pre-T neoplasm is associated with what other parts of the body

A
  • airway compression ( medastinal mass)

- testicular involvement

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9
Q

what do ALL cells look like? what do they not have?

A

high N:C ratio
irregular nucleus
- no MPO

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10
Q

what do both precursor B- and T- cell neoplasms have on their immunophenotype

A

CD34

TdT

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11
Q

CD 19, 22, 10

A

Immunophenotpye of pre-B

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12
Q

CD 1a, 2, 5, 7

A

Immunophenotpye of pre-T

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13
Q

what are the two cytogenetic abnormality for Pre-B? who usually gets which one

A

T (12;21) children

T (9;22) adults

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14
Q

ALL prognosis for children

A

good

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15
Q

ALL prognosis for adults

A

not good

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16
Q

how is ALL treated

A

aggressive chemo plus CNS prophylaxis

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17
Q

what has improved prognosis in t(9;22) in pre-B ALL

A

BCR-ABL Tyrosine kinase inhibitor therapy

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18
Q

what is the most common leukemia of adults in western world

A

CLL/SLL

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19
Q

CLL/SLL which one is leukemic and which one is lymphomatous

A

CLL: leukemic
SLL: lymphomatous

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20
Q

Who usually presents with CLL/SLL

A

over 50 years of age

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21
Q

what are symptoms for CLL/SLL

A

a symptomatic

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22
Q

name 3 complications of CLL/SLL

A
  1. hypogammaglobulinemia
  2. autoimmune hemolytic anemia
  3. Richter transformation
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23
Q

what does the histo slide look like with CLL

A

smudge cells
hyperclumped nuclear chromatin
lymphadenopathy

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24
Q

what does the histo slide look like for SLL

A

proliferation centers mimic germinal centers

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25
Q

CD 19,5,23

CD 20- dim surface light chain restricted

A

immunophenotype for CLL/SLL

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26
Q

what is Richter transformation

A

transforms to large B-cell lymphoma

-enlarging lymph node or spleen

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27
Q

what is the prognosis for CLL/SLL

A

poor, die from other problems

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28
Q

Follicular lymphoma strongly associated with

A

translocations involving BCL2

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29
Q

Characteristic of lymph nodes for follicular lymphoma

A

painless lymphadenopathy

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30
Q

what’s the involvement of BM and PB for follicular lymphoma

A

BM involved

PB not

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31
Q

what are 2 cell types in follicluar lymphoma in lypms

A

centrocytes: small cleaved cells
centroblasts: larger cells; count for grading

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32
Q

what is seen in the spleem for follicular lymphoma

A

white pulp; portal tracts

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33
Q

CD 19, 20, 10
surface light chain
BCL2
blc6

A

what is the immunophenotype for follicular lymphoma

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34
Q

what is the cytogenetics of follicular lymphoma? what is on each chromosome

A

t ( 14;18)
14- IgH
18- BCL2

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35
Q

prognosis for follicular lymphoma?

A

incurable

- transformation to diffuse large b cell lymphoma

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36
Q

for mantle cell lymphoma, tumor cells express what

A

CD 5

over express cyclin D1

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37
Q

what is the cytogenetics of mantle cell lympohma

A

t (11;14)

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38
Q

what is the PB and MB involvement for mantle cell lymphoma

A

PB: varies
BM: most involvement

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39
Q

what is characteristic of the lymph nodes in mantle cell lymphoma

A

painless, generalized lemphadenopathy

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40
Q

mantle cell lymphoma involves what other organ disorder

A

lymphomatoid polyposis= GI invovlment

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41
Q

CD 19, 20, 5

bright surface light chain

A

what is the immunophenotype for mantle cell lymphoma

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42
Q

Immunophenotype for mantle cell lymphoma is negative for

A

CD 23

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43
Q

what is the cytogenetics for mantle cell lymphoma? what do each chromosome express?

A

t(11;14)

11: cyclin D1
14: IgH

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44
Q

what does increased expression of cyclin D1 in mantle cell lymphoma do to cell

A

promotes G1 to S phase

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45
Q

what is the prognosis for mantle cell lymphoma

A

not curable

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46
Q

what is the treatment for mantle cell lymphoma

A

Retuximab and chemotherapy

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47
Q

marginal zone lymphoma is formed by

A

post-germinal center memory B cells

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48
Q

marginal zone lymphoma is involved in what type of disorders

A

chronic inflammatory

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49
Q

histo what does morginal zone lymphoma have?

A

monocytoid B-cells

plasmacytoid celld

50
Q

CD19, 20

surface light chain

A

Immunophenotype for marginal zone lymphoma

51
Q

what is the cytogenetics for marginal zone lyphoma

A

t ( 11;18) or t (11;14)

52
Q

what does lymphoplasacytic lymphoma secrete

A

monoclonal IgM

-Waldenstorm macroglobulinemia ( hyper viscosity)

53
Q

what are symptoms for waldenstorm’s macroglobulinemia from the IgM

A

visual
neurologic
bleeding
cryoglobulinemia (IgM precipitates at low temp)

54
Q

how do you alleviate symptoms of IgM from Waldenstorm’s macroglobulinemia

A

plasmapheresis

55
Q

lymphoplasmacytic lymphoma is a mutation in what

A

MYD88

56
Q

Hairy cell leukemia is a mutation in what

A

BRAF

57
Q

what does hairy cell leukemia involve

A

PB
BM
red pulp spleen

58
Q

clinical features for hairy cell leukemia

A

splenomegaly
pancytopenia
monocytopenia
cytopenias

59
Q

what’s the bone tap for hairy cell leukemia

A

dry tap

60
Q

what is the histo for hairy cell leukemia

A

diffuse fried eggs

61
Q

CD 19, 20
surface light chain
CD 11c, 25, 103 ( diagnostic)

A

Immunophenotype for hairy cell leukemia

62
Q

what do both precursor B- and T- cell neoplasms not have on their immunophenotype

A

surface light chain

63
Q

what do plasma cell neoplasms secrete

A

M-component

Bence Jones protien

64
Q

what are 3 important things for multiple myelome

A

M-protein in blood or urine
clonal plasma cells
end organ damage

65
Q

CD 38 / 56

intracytoplasmic monoclonal light chain

A

what is the immunophenotpye for plasma cell neoplasms

66
Q

what is the CBC for multiple myeloma

A

anemia sometimes pancytopenia

67
Q

metabolic panal for multiple myeloma

A

hypercalcemia

increased creatine

68
Q

what does monoclonal protein mean in multiple meyloma

A

one class of Ig will be elevated rest will be decreased

69
Q

what does the radiology x-ray look like for multiple myeloma

A

bony lytic lesions

70
Q

tumor cells in multiple meyoloma produce what

A

IL-6 ( growth factor for tumor cells)

71
Q

in mulitple myeloma tumor cells have what influence

A

activation of osteoclasts

inhibition of osteoblasts

72
Q

for multiple myeloma what is required for diagnosis

A

bone marrow

73
Q

what is seen in the peripheral blood for multiple myeloma

A

roulex due to increased protein

-excess immunoglobulins

74
Q

MGUS what is seen in blood and urine

A

BJ protein and M protein levels

75
Q

plasmacytoma

A

solitary bone or soft tissue clonal plasma cell masses with no evidence of marrow or organ disease

76
Q

1% of MGUS develop what after one year

A

multiple myeloma

77
Q

where does plasmacytoma most likely occur in bone and soft tissue

A

spine

lung and oronasopharynx

78
Q

what is plasmacytoma treated

A

local field radiation therapy

79
Q

Diffuse large B cell lymphoma define

A

high grade

heterogenous group

80
Q

what virus is associated with diffuse large B cell lymphoma

A

EBV

81
Q

what are clinical features of the disease diffuse large B-cell Lymphoma

A

rapidly enlarging
B-type symptoms
BM involvement later

82
Q

what is the most common form of NHL

A

diffuse large B-cell Lymphoma

83
Q

what is the histology for DLBCL

A

1-3 nuclei, pale cytoplasm

indistinct cell borders

84
Q

CD 19, 20

surface light chain restriction

A

immunophenotype for DLBCL

85
Q

what are the cytogenetic mutations for DLBCL

A

BCL6
BCL2
MYC
double hit lymphomas

86
Q

what is the role of BCL6

A

represses normal B-cell differentiation

87
Q

define Burkitt Lymphoma

A

high grade

88
Q

what are the 3 different types of Burkitt Lymphomas and where do some of them occur

A

endemic EBV ( african Malaria belt)
sporadic ( US children)
HIV

89
Q

what is the clincal menifestation for the endemic burkitt lymphoma

A

mandibular mass

abdominal viscera

90
Q

what is the clinical manifestation for sporadic burkitt lymphoma

A

abdominal mass in ileocecum and peritoneum

91
Q

what are two broad clinical features of Burkitt Lymphoma

A

Tumor Lysis syndrome

CNS disease

92
Q

what is the buzzword for Burkitt lymphoma

A

Starry sky

93
Q

what are two characteristics of Burkitt lymphoma cells

A

high mitotic activity

apoptotic cells

94
Q

why does Burkitt lymphoma cell grow so fast

A

TRANSLOCATION OF MYC

95
Q

CD 19, 20, 10
surface light chain restriction
bcl6

A

what is the immunophenotype for Burkitt Lymphoma

96
Q

what are the translocations for Burkitt lymphoma

A

t (8;14)
t ( 2;8)
t ( 8;22)

97
Q

treatment for Burkitt lymphoma

A

aggresive chemotherapy with intrathecal CNS chemotherapy

98
Q

PCR is used to identify what in mature T cell neoplasms

A

id monoclonal rearrangements of at least one TCR locus

99
Q

define peripheral t cell lymphoma, not otherwise specified ( PTCL-NOS)

A

T cell counterpart of DLBCL

100
Q

clinical features for PTCL-NOS

A

generalized lymphadoenopathy
eosinophilia
pruritis, fever, weight loss

101
Q

prognosis for PTCL-NOS

A

worse than B

102
Q

immunophenotype for PTCL-NOS

A

mature T cell phenotype

103
Q

define anaplastic large cell lymphoma

A

translocation involving ALK ( behave like tyrosine kinase) gene on 2p23

104
Q

clinical presentation for anaplastic large cell lymphoma

A

advanced stage with adenopathy
extranodal infiltrates
BM involvement

105
Q

what is the hallmark cells for anaplastic large cell lympohoma

A

horsehoe or wreath shaped nuclei

106
Q

what is the sinusoidal pattern of anaplastic large celll lympohma

A

neoplastic cells around venules in sinuses of node

107
Q

what is the prognosis for kids ALCL lymphoma +

A

good with chemo

108
Q

prognosis for adutls ALCL lympohma + or -

A

negative less favorable

109
Q

define Adult T-cell leukemia/lymphoma

A

CD4 t cells infected with retrovirus HTLV-1

110
Q

what are two different presentations for ATCL and prognosis

A

leukmic = rapidly progressive

skin localized - less aggresive

111
Q

histo cells of ATCL

A

flower/cloverleaf cells

112
Q

immunophenotype for ATCL

A

CD4+/CD7-

HTVL-1+

113
Q

Mycosis Fungoides/Sezary syndrome (MF/SS)

A

different manifestations of tumor of CD4+ helper T cells that home to the skin

114
Q

clinical features of MF

A

patch
plaque
tumor

115
Q

clinical features of SS

A

generalized exfoliative erythroderma plus leukemia

116
Q

histo what does neoplastic and sezary cell look like

A

neoplast: CEREBRIFORM nuclear contours
sezary: neoplastic in BLOOD

117
Q

Large granular lymphocytic leukemia define

A

T cell type and NK

118
Q

Large granular lymphocytic leukemia associated disease

A

Felty syndrome

119
Q

Large granular lymphocytic leukemia what happens

A

neutropenia with associated decreased myeloid maturation in BM

120
Q

Large granular lymphocytic leukemia pathology

A

STAT 3 mutations

large granular lymphocytes