B3-041 Introduction to Lymphoid Disorders Flashcards
(158 cards)
1
Q
lymphoid neoplasms are derived from
A
precursor cells
mature B cells
or mature T/NK cells
2
Q
lymphoid neoplasms present primarily in
A
bone marrow
lymph nodes
extranodal tissue
3
Q
lymphoid neoplasms are classified by
A
cell of origin
developmental stage of transformed cell
clinical, immunophenotypic, genetic, and molecular findings
4
Q
primarily bone marrow and blood involvement
A
leukemia
5
Q
myeloid or lymphoid origin
A
leukemia
6
Q
may secondarily involve lymph nodes and solid tissues
A
leukemia
7
Q
primarily lymph node or solid tissue involvment
A
lymphoma
8
Q
lymphoid origin
A
lymphoma
9
Q
may secondarily involve bone marrow and blood (leukemic phase)
A
lymphoma
10
Q
chronic lymphocytic luekemia =
A
small lymphocytic lymphoma
11
Q
acute lymphoblastic leukemia =
A
lymphoblastic lymphoma
12
Q
primarily immature cells [blasts]
A
acute
13
Q
if untreated, rapid progression and fatal in weeks to months
A
acute
14
Q
primarily mature or maturing cells
A
chronic
15
Q
if untreated, slow progression and fatal in months to years
A
chronic
16
Q
B cell neoplasms make up ____% of all lymphoid neoplasms
A
80
17
Q
T/NK cell neoplasms make up ___% of all lymphoid neoplasms
A
20
18
Q
[2] most frequently occurring non-hodgkin lymphomas
A
diffuse large B cell
folllicular lymphoma
19
Q
mature B neoplasms of the bone marrow/blood [3]
A
CLL/SLL
hairy cell leukemia
Burkitt
20
Q
mature B neoplasms of the lymph node [5]
A
CLL/SLL
follicular lymphoma
diffuse-large B cell lymphoma
mantle cell lymphoma
Burkitt
21
Q
mature B neoplasms, extranodal [2]
A
diffuse large B cell lymphoma
marginal zone lymphoma
22
Q
mature T neoplasms of bone marrow/blood [2]
A
adult T cell leukemia
mycosis fungoides
23
Q
mature T neoplasms of the lymph node [3]
A
peripheral T cell lymphoma
anaplastic large cell lymphoma
adult-t cell lymphoma
24
Q
mature T neoplasms, extranodal [1]
A
mycosis fungoides
25
CD19, 20
monoclonal surface immunoglobin
generic immunophenotype for
mature B neoplasms
26
CD2, 3, 5, 7 (maybe 4 or 8)
are generic immunophenotype markers for
mature T neoplasms
27
monoclonal rearrange immunoglobin genes
mature B neoplasms
28
translocations involving chr. 14
mature B neoplasms
29
monoclonal rearranged TCR receptor genes
mature T neoplasms
30
small lymphoid neoplasms tend to be [more/less] aggressive
less
31
diffuse
32
small cell
33
nodular
34
large cell
35
older adults; most common leukemia in US
CLL/SLL
36
small mature lymphocytes in blood, bone marrow
CLL
37
diffuse infiltrate of small lymphocytes in lymph nodes
SLL
38
variable anemia
thrombocytopenia
lymphadenopathy
maybe autoimmune anemia
CLL/SLL
39
CLL/SLL
smudge cells
40
CLL/SLL marrow
41
CLL/SLL
marrow
42
CD19+
**CD20+**
**CD5+**
CD10-
**sIg + (clonal)**
CLL/SLL
43
which is more favorable: unmutated or mutated CLL/SLL
unmutated
| no somatic hypermutation, occurred further up cell line
44
favorable deletion for CLL/SLL
13q
45
unfavorable deletions for CLL/SLL
| 3
11q, 17p, trisomy 12
46
which neoplasm can undergo Richter transformation?
CLL/SLL
| transforms to large cell lymphoma with increased lymphadenopathy, <1 y.
47
middle aged/older adults
hepatosplenomegaly
pancytopenia
infections
hairy cell leukemia
48
lymphocytes with **villous** **projections**
hairy cell leukemia
49
CD19+
CD20+
**CD10-**
**CD5-**
**sIg+ (clonal)**
**CD11c+**
**CD25+**
***CD103+***
***Annexin A1***
## Footnote
italics are specific markers
hairy cell leukemia
50
BRAF mutation
hairy cell leukemia
51
hairy cell leukemia
52
common in middle aged/older adults
lymphadenopathy, hepatosplenomegaly
follicular lymphoma
53
modular infiltrate of **small cleaved lymphocytes** in lymph nodes
diminshed mantel zone
follicular lymphoma
54
CD19+
CD20+
***CD10+***
CD5-
sIg (clonal)
Follicular lymphoma
55
t(14;18) is associated with
follicular lymphoma
| causes overexpression of Bcl-2 protein, prevent apoptosis
56
follicular lymphoma
57
follicular lymphoma
58
middle age/older adults
lymphadenopathy
hepatosplenomegaly
often advanced stage
Mantle cell lymphoma
59
**nodular or diffuse infiltrate** of small **lymphocytes** in lymph nodes
mantle cell lymphoma
60
CD19+
Cd20+
CD10-
***CD5+***
sIg (clonal)
***Cyclin D1+***
mantle cell lymphoma
61
t(11;14)
mantle cell lymphoma
| overexpression of cyclin D
distinguishes from CML
62
prognosis of mantle cell lymphoma
poor; 3-4 years
63
association with immune reactions and chronic infections
Marginal Zone lymphoma
## Footnote
H. pylori
64
infiltrate of lymphoid cells into epithelium and expanded marginal zone
marginal zone lymphoma
65
CD19+
CD20+
**CD10-**
**CD5-**
**sIg+ (clonal)**
marginal zone lymphoma
66
middle/older adults
most common lymphoma
lymphadenopathy
hepatosplenomegaly
frequently extranodal
large B cell lymphoma
67
**diffuse** infiltrate of **large lymphocytes** in lymph nodes
large B cell lymphoma
68
CD19+
CD20+
**CD10+**
**CD5-**
**sIg+ clonal**
large B cell lymphoma
69
sometimes associated with EBV and HHV8
large B cell lymphoma
70
may be positive MYC translocation
large B cell lymphoma
| more aggresssive is MYC translocation positive
71
large B cell
72
rapidly progressive tumor mass
tumor lysis syndrome
| extremely high growth fraction
Burkitt
73
extremely responsive to treatment, causing tumor lysis syndrome
Burkitt
| hydration and kidney protection
74
form of Burkitt in equatorial Africa
| primarily affects children
endemic
75
>95% associated with EBV
endemic Burkitt lymphoma
76
form of Burkitt in US
| involves children and adults
sporadic
77
15-20% associated with EBV
sporadic lymphoma
78
25% associated with EBV
| Burkitts
HIV associated Burkitts
79
Burkitt types: best to worse response to therapy
endemic>sporadic>HIV associated
80
**diffuse** infiltrate of **medium sized lymphocytes**
| high mitotic rate; tingible body macrophages
Burkitt
81
CD19+
CD20+
**CD10+**
**CD5-**
sIg
***TdT-***
Burkitt
82
t(8;14)
[variants t(2;8), t(822)]
Burkitt
| MYC oncogene translocation
83
Burkitt prognosis
highly aggressive but 60-90% cure rate with therapy
84
Burkitt
| starry sky
85
older adults
cutaneuous patch, plaque, and nodule stages
mycosis fungoides
Sezary Syndrome
86
dermal infitrate of atypical lymphocytes with **epidermotropism and Pautrier microabscesses**
Mycosis fungoides
87
circulating lymphoid cells with **cerebriform nuclei and erythroderma**
Sezary Syndrome
88
CD4+
Mycosis fungoides/Sezary Syndrome
89
mycosis fungoides
90
mycosis fungoides
91
Sezary syndrome
| delicately folded nuclei, look like gyri and sulci of brain --> cerebrif
92
middle age/older adults
lymphadenopathy
skin lesions
hepatosplenomegaly
lymphocytosis
hypercalcemia
adult T cell leukemia
93
endemic in Japan, Carribean, West Africa
adult T cell lymphoma
94
caused by HTLV-1
adult T cell lymphoma
95
prognosis Adult T cell lymphoma
poor, especially in leukemic phase
96
CD30+
anaplastic large cell lymphoma
97
t(2;5) rearrangement of ALK indicates good prognosis of
anaplastic large cell lymphoma
| **poor prognosis if ALK-
98
lymphadenopathy
fever, pruritus, weight loss
**eosinophilia**
peripheral T cell lymphoma
99
prognosis: peripheral T cell lymphoma
poor
20-30% 5 year survival
100
Bimodal age distribution (15-35, >50)
most common malignancy in 10-30 age group
Hodgkin Lymphoma
101
B symptoms
fever, night sweats, weight loss
102
lymphadenopathy
splenomegaly
B symptoms
immune dysfunction
| possible relation to EBV
Hodgkin Lymphoma
103
Reed Sternberg cells in a polymorphous cellular background
classical Hodgkin Lymphoma
104
no reed sternburg cells; large B cells in a background of small lymphocytes
nodular lymphocyte predominant Hodgkin lymphoma
105
Hodgkin lymphoma
| Reed sternburg cells
106
Hodgkin
107
heterogenous cell population
hodgkin
108
homogenous cell population
non-hodgkin
109
often localized to single nodal group
hodgkin
110
spread along continuous nodal groups
hodgkin
111
frequently involve multiple nodal groups
non-hodgkins
112
spread to any site in unpredictable manner
non-hodgkin
113
waldeyer ring and mesenteric nodes commonly involved
non-Hodgkins
114
**CD15+**
**CD30+**
CD20+
hodgkin
115
mutated germinal center B cells that escape apoptotic death
Hodgkin
116
long term hodgkin's survivors have an increased risk of
secondary malignancy
117
CD20+ (dim)
CD5+
**CD23+**
**FMC7-**
CLL/SLL
118
CD20+
CD11C+
CD25+
CD103+
Annexin A1
BRAF
hairy cell
119
CD20+
MALT
| marginal zone
120
CD20+
CD10+
BCL2+
t(14;18)
Follicular
121
CD20+
CD5+
**CD23-**
**FMC7+**
Cyclin D1 +
t(11;14)
mantle cell
122
non neoplastic proliferation of lymphocytes can be caused by
viral infections
acute bacterial infections
chronic bacterial infections
atypical lymphocytes
123
non-neoplastic lymphadenopathy can be caused by
follicular hyperplasia
paracortical hyperplasia
sinus histiocytosis
124
atypical lymphocytes
| viral infection
125
atypical lymphocytes
no evidence of clonality
increase in CD8+
think viral infection, mononucleosis
126
granulocytic leukocytosis with left shift
CML
| as well as bacterial infections (pneumoia, strep)
127
blasts
AML
128
polymorphous infiltrate including lymphocytes, plasma cell, eosinophils, macrophages
Hodgkin
129
Reed Sternburg cell markers
CD15
CD30
| Hodgkin
130
myeloid cell markers
CD13
CD33
CD117
myeloperoxidase
131
hairy cell leukemia markers
CD103
CD25
CD11c
132
germinal center cells in lymph node with reactive follicular hyperplasia are characterized by
lack of Bcl-2 expression
133
adult t cell lymphoma is endemic to
japan, west africa, carribean
134
derived from B cells
rarely involves CNS
hodgkin
135
most common in young adults with second peak after 50
hodgkin
136
painless cervical or axillary lymph nodes
hodgkin
137
B cell lymphomas comprise ...% of non-hodgkin lymphomas
80
138
extranodal
non-hodgkins lymphoma
139
required for diagnosis of hodgkin
reed sternburg cells
140
presence of somatic mutations in CLL is
favorable
141
no somatic mutations in CLL is
unfavorable
142
CD20+
CD10-
CD5+
CLL
143
therapy of CLL is
not needed unless symptomatic
144
indolent with a mean survival of several years
CLL
145
smudge cells can be indicative of
CLL
146
CLL/SLL is derived from
mature B cells
147
the overexpression of Bcl-2 in follicular lymphoma is due to
t(14;18)
148
bimodal age distribution and most common lymphoma is young adults
hodgkin
149
cases with t(2;5) ALK translocation have more favorable prognosis
anaplastic large cell lymphoma
150
if ALK+, anaplastic large cell lymphoma can be treated with
small molecule inhibitor of ALK
151
ALK- is unfavorable for
anaplastic large cell lymphoma
152
aggressive course despite small lymphocyte size, CD20+, CD5+
mantle cell
153
MYC translocation and frequent extranodal presentation in endemic area
burkitt
154
cutaneous patches, plaques, or nodules that histologically show atypical T cells in dermis and epidermis
mycosis fungoides
155
most common lymphoma in US, both nodal and extranodal presentations common, aggressive
diffuse large B cell
156
circulating lymphoid cells with villous cytoplasmic projections; BRAF mutation
hairy cell leukemia
157
reed sternburg cells in polymorphous background
hodgkin
158
aggressive, growing very rapidly with nearly 100% growth fraction
Burkitt
