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IBI Test 3 > B3-041 Introduction to Lymphoid Disorders > Flashcards

B3-041 Introduction to Lymphoid Disorders Flashcards

1
Q

lymphoid neoplasms are derived from

A

precursor cells
mature B cells
or mature T/NK cells

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2
Q

lymphoid neoplasms present primarily in

A

bone marrow
lymph nodes
extranodal tissue

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3
Q

lymphoid neoplasms are classified by

A

cell of origin
developmental stage of transformed cell
clinical, immunophenotypic, genetic, and molecular findings

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4
Q

primarily bone marrow and blood involvement

A

leukemia

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5
Q

myeloid or lymphoid origin

A

leukemia

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6
Q

may secondarily involve lymph nodes and solid tissues

A

leukemia

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7
Q

primarily lymph node or solid tissue involvment

A

lymphoma

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8
Q

lymphoid origin

A

lymphoma

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9
Q

may secondarily involve bone marrow and blood (leukemic phase)

A

lymphoma

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10
Q

chronic lymphocytic luekemia =

A

small lymphocytic lymphoma

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11
Q

acute lymphoblastic leukemia =

A

lymphoblastic lymphoma

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12
Q

primarily immature cells [blasts]

A

acute

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13
Q

if untreated, rapid progression and fatal in weeks to months

A

acute

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14
Q

primarily mature or maturing cells

A

chronic

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15
Q

if untreated, slow progression and fatal in months to years

A

chronic

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16
Q

B cell neoplasms make up ____% of all lymphoid neoplasms

A

80

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17
Q

T/NK cell neoplasms make up ___% of all lymphoid neoplasms

A

20

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18
Q

[2] most frequently occurring non-hodgkin lymphomas

A

diffuse large B cell
folllicular lymphoma

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19
Q

mature B neoplasms of the bone marrow/blood [3]

A

CLL/SLL
hairy cell leukemia
Burkitt

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20
Q

mature B neoplasms of the lymph node [5]

A

CLL/SLL
follicular lymphoma
diffuse-large B cell lymphoma
mantle cell lymphoma
Burkitt

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21
Q

mature B neoplasms, extranodal [2]

A

diffuse large B cell lymphoma
marginal zone lymphoma

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22
Q

mature T neoplasms of bone marrow/blood [2]

A

adult T cell leukemia
mycosis fungoides

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23
Q

mature T neoplasms of the lymph node [3]

A

peripheral T cell lymphoma
anaplastic large cell lymphoma
adult-t cell lymphoma

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24
Q

mature T neoplasms, extranodal [1]

A

mycosis fungoides

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25
Q

CD19, 20
monoclonal surface immunoglobin

generic immunophenotype for

A

mature B neoplasms

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26
Q

CD2, 3, 5, 7 (maybe 4 or 8)

are generic immunophenotype markers for

A

mature T neoplasms

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27
Q

monoclonal rearrange immunoglobin genes

A

mature B neoplasms

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28
Q

translocations involving chr. 14

A

mature B neoplasms

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29
Q

monoclonal rearranged TCR receptor genes

A

mature T neoplasms

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30
Q

small lymphoid neoplasms tend to be [more/less] aggressive

A

less

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31
Q
A

diffuse

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32
Q
A

small cell

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33
Q
A

nodular

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34
Q
A

large cell

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35
Q

older adults; most common leukemia in US

A

CLL/SLL

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36
Q

small mature lymphocytes in blood, bone marrow

A

CLL

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37
Q

diffuse infiltrate of small lymphocytes in lymph nodes

A

SLL

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38
Q

variable anemia
thrombocytopenia
lymphadenopathy
maybe autoimmune anemia

A

CLL/SLL

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39
Q
A

CLL/SLL

smudge cells

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40
Q
A

CLL/SLL marrow

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41
Q
A

CLL/SLL

marrow

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42
Q

CD19+
CD20+
CD5+
CD10-
sIg + (clonal)

A

CLL/SLL

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43
Q

which is more favorable: unmutated or mutated CLL/SLL

A

unmutated

no somatic hypermutation, occurred further up cell line

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44
Q

favorable deletion for CLL/SLL

A

13q

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45
Q

unfavorable deletions for CLL/SLL

3

A

11q, 17p, trisomy 12

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46
Q

which neoplasm can undergo Richter transformation?

A

CLL/SLL

transforms to large cell lymphoma with increased lymphadenopathy, <1 y.

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47
Q

middle aged/older adults
hepatosplenomegaly
pancytopenia
infections

A

hairy cell leukemia

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48
Q

lymphocytes with villous projections

A

hairy cell leukemia

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49
Q

CD19+
CD20+
CD10-
CD5-
sIg+ (clonal)
CD11c+
CD25+
CD103+
Annexin A1

italics are specific markers

A

hairy cell leukemia

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50
Q

BRAF mutation

A

hairy cell leukemia

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51
Q
A

hairy cell leukemia

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52
Q

common in middle aged/older adults
lymphadenopathy, hepatosplenomegaly

A

follicular lymphoma

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53
Q

modular infiltrate of small cleaved lymphocytes in lymph nodes
diminshed mantel zone

A

follicular lymphoma

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54
Q

CD19+
CD20+
CD10+
CD5-
sIg (clonal)

A

Follicular lymphoma

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55
Q

t(14;18) is associated with

A

follicular lymphoma

causes overexpression of Bcl-2 protein, prevent apoptosis

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56
Q
A

follicular lymphoma

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57
Q
A

follicular lymphoma

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58
Q

middle age/older adults
lymphadenopathy
hepatosplenomegaly
often advanced stage

A

Mantle cell lymphoma

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59
Q

nodular or diffuse infiltrate of small lymphocytes in lymph nodes

A

mantle cell lymphoma

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60
Q

CD19+
Cd20+
CD10-
CD5+
sIg (clonal)
Cyclin D1+

A

mantle cell lymphoma

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61
Q

t(11;14)

A

mantle cell lymphoma

overexpression of cyclin D
distinguishes from CML

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62
Q

prognosis of mantle cell lymphoma

A

poor; 3-4 years

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63
Q

association with immune reactions and chronic infections

A

Marginal Zone lymphoma

H. pylori

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64
Q

infiltrate of lymphoid cells into epithelium and expanded marginal zone

A

marginal zone lymphoma

65
Q

CD19+
CD20+
CD10-
CD5-
sIg+ (clonal)

A

marginal zone lymphoma

66
Q

middle/older adults
most common lymphoma
lymphadenopathy
hepatosplenomegaly
frequently extranodal

A

large B cell lymphoma

67
Q

diffuse infiltrate of large lymphocytes in lymph nodes

A

large B cell lymphoma

68
Q

CD19+
CD20+
CD10+
CD5-
sIg+ clonal

A

large B cell lymphoma

69
Q

sometimes associated with EBV and HHV8

A

large B cell lymphoma

70
Q

may be positive MYC translocation

A

large B cell lymphoma

more aggresssive is MYC translocation positive

71
Q
A

large B cell

72
Q

rapidly progressive tumor mass

tumor lysis syndrome

extremely high growth fraction

A

Burkitt

73
Q

extremely responsive to treatment, causing tumor lysis syndrome

A

Burkitt

hydration and kidney protection

74
Q

form of Burkitt in equatorial Africa

primarily affects children

A

endemic

75
Q

> 95% associated with EBV

A

endemic Burkitt lymphoma

76
Q

form of Burkitt in US

involves children and adults

A

sporadic

77
Q

15-20% associated with EBV

A

sporadic lymphoma

78
Q

25% associated with EBV

Burkitts

A

HIV associated Burkitts

79
Q

Burkitt types: best to worse response to therapy

A

endemic>sporadic>HIV associated

80
Q

diffuse infiltrate of medium sized lymphocytes

high mitotic rate; tingible body macrophages

A

Burkitt

81
Q

CD19+
CD20+
CD10+
CD5-
sIg
TdT-

A

Burkitt

82
Q

t(8;14)
[variants t(2;8), t(822)]

A

Burkitt

MYC oncogene translocation

83
Q

Burkitt prognosis

A

highly aggressive but 60-90% cure rate with therapy

84
Q
A

Burkitt

starry sky

85
Q

older adults
cutaneuous patch, plaque, and nodule stages

A

mycosis fungoides
Sezary Syndrome

86
Q

dermal infitrate of atypical lymphocytes with epidermotropism and Pautrier microabscesses

A

Mycosis fungoides

87
Q

circulating lymphoid cells with cerebriform nuclei and erythroderma

A

Sezary Syndrome

88
Q

CD4+

A

Mycosis fungoides/Sezary Syndrome

89
Q
A

mycosis fungoides

90
Q
A

mycosis fungoides

91
Q
A

Sezary syndrome

delicately folded nuclei, look like gyri and sulci of brain –> cerebrif

92
Q

middle age/older adults
lymphadenopathy
skin lesions
hepatosplenomegaly
lymphocytosis
hypercalcemia

A

adult T cell leukemia

93
Q

endemic in Japan, Carribean, West Africa

A

adult T cell lymphoma

94
Q

caused by HTLV-1

A

adult T cell lymphoma

95
Q

prognosis Adult T cell lymphoma

A

poor, especially in leukemic phase

96
Q

CD30+

A

anaplastic large cell lymphoma

97
Q

t(2;5) rearrangement of ALK indicates good prognosis of

A

anaplastic large cell lymphoma

**poor prognosis if ALK-

98
Q

lymphadenopathy
fever, pruritus, weight loss
eosinophilia

A

peripheral T cell lymphoma

99
Q

prognosis: peripheral T cell lymphoma

A

poor
20-30% 5 year survival

100
Q

Bimodal age distribution (15-35, >50)
most common malignancy in 10-30 age group

A

Hodgkin Lymphoma

101
Q

B symptoms

A

fever, night sweats, weight loss

102
Q

lymphadenopathy
splenomegaly
B symptoms
immune dysfunction

possible relation to EBV

A

Hodgkin Lymphoma

103
Q

Reed Sternberg cells in a polymorphous cellular background

A

classical Hodgkin Lymphoma

104
Q

no reed sternburg cells; large B cells in a background of small lymphocytes

A

nodular lymphocyte predominant Hodgkin lymphoma

105
Q
A

Hodgkin lymphoma

Reed sternburg cells

106
Q
A

Hodgkin

107
Q

heterogenous cell population

A

hodgkin

108
Q

homogenous cell population

A

non-hodgkin

109
Q

often localized to single nodal group

A

hodgkin

110
Q

spread along continuous nodal groups

A

hodgkin

111
Q

frequently involve multiple nodal groups

A

non-hodgkins

112
Q

spread to any site in unpredictable manner

A

non-hodgkin

113
Q

waldeyer ring and mesenteric nodes commonly involved

A

non-Hodgkins

114
Q

CD15+
CD30+
CD20+

A

hodgkin

115
Q

mutated germinal center B cells that escape apoptotic death

A

Hodgkin

116
Q

long term hodgkin’s survivors have an increased risk of

A

secondary malignancy

117
Q

CD20+ (dim)
CD5+
CD23+
FMC7-

A

CLL/SLL

118
Q

CD20+
CD11C+
CD25+
CD103+
Annexin A1
BRAF

A

hairy cell

119
Q

CD20+

A

MALT

marginal zone

120
Q

CD20+
CD10+
BCL2+
t(14;18)

A

Follicular

121
Q

CD20+
CD5+
CD23-
FMC7+
Cyclin D1 +
t(11;14)

A

mantle cell

122
Q

non neoplastic proliferation of lymphocytes can be caused by

A

viral infections
acute bacterial infections
chronic bacterial infections
atypical lymphocytes

123
Q

non-neoplastic lymphadenopathy can be caused by

A

follicular hyperplasia
paracortical hyperplasia
sinus histiocytosis

124
Q
A

atypical lymphocytes

viral infection

125
Q

atypical lymphocytes
no evidence of clonality
increase in CD8+

A

think viral infection, mononucleosis

126
Q

granulocytic leukocytosis with left shift

A

CML

as well as bacterial infections (pneumoia, strep)

127
Q

blasts

A

AML

128
Q

polymorphous infiltrate including lymphocytes, plasma cell, eosinophils, macrophages

A

Hodgkin

129
Q

Reed Sternburg cell markers

A

CD15
CD30

Hodgkin

130
Q

myeloid cell markers

A

CD13
CD33
CD117
myeloperoxidase

131
Q

hairy cell leukemia markers

A

CD103
CD25
CD11c

132
Q

germinal center cells in lymph node with reactive follicular hyperplasia are characterized by

A

lack of Bcl-2 expression

133
Q

adult t cell lymphoma is endemic to

A

japan, west africa, carribean

134
Q

derived from B cells
rarely involves CNS

A

hodgkin

135
Q

most common in young adults with second peak after 50

A

hodgkin

136
Q

painless cervical or axillary lymph nodes

A

hodgkin

137
Q

B cell lymphomas comprise …% of non-hodgkin lymphomas

A

80

138
Q

extranodal

A

non-hodgkins lymphoma

139
Q

required for diagnosis of hodgkin

A

reed sternburg cells

140
Q

presence of somatic mutations in CLL is

A

favorable

141
Q

no somatic mutations in CLL is

A

unfavorable

142
Q

CD20+
CD10-
CD5+

A

CLL

143
Q

therapy of CLL is

A

not needed unless symptomatic

144
Q

indolent with a mean survival of several years

A

CLL

145
Q

smudge cells can be indicative of

A

CLL

146
Q

CLL/SLL is derived from

A

mature B cells

147
Q

the overexpression of Bcl-2 in follicular lymphoma is due to

A

t(14;18)

148
Q

bimodal age distribution and most common lymphoma is young adults

A

hodgkin

149
Q

cases with t(2;5) ALK translocation have more favorable prognosis

A

anaplastic large cell lymphoma

150
Q

if ALK+, anaplastic large cell lymphoma can be treated with

A

small molecule inhibitor of ALK

151
Q

ALK- is unfavorable for

A

anaplastic large cell lymphoma

152
Q

aggressive course despite small lymphocyte size, CD20+, CD5+

A

mantle cell

153
Q

MYC translocation and frequent extranodal presentation in endemic area

A

burkitt

154
Q

cutaneous patches, plaques, or nodules that histologically show atypical T cells in dermis and epidermis

A

mycosis fungoides

155
Q

most common lymphoma in US, both nodal and extranodal presentations common, aggressive

A

diffuse large B cell

156
Q

circulating lymphoid cells with villous cytoplasmic projections; BRAF mutation

A

hairy cell leukemia

157
Q

reed sternburg cells in polymorphous background

A

hodgkin

158
Q

aggressive, growing very rapidly with nearly 100% growth fraction

A

Burkitt

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