Lecture 149 Flashcards
(108 cards)
1
Q
Proliferations of white cells, typically lymphocytes, usually present as discrete tissue masses, AKA
A
Lymphoma
2
Q
2/3 of NHLs and all HLs present with ____?
A
Enlarged nontender lymph nodes
3
Q
The vast majority of lymphoid neoplasms are of what origin?
A
B-cell
4
Q
Lymphoid neoplasms are often associated with abnormalities in what system?
A
Immune abnormalities (loss of protective immunity or autoimmunity)
5
Q
What is the spread pattern of Hodgkin lymphoma?
A
Spreads in an orderly, step-wise fashion
6
Q
What is the spread pattern of Non-Hodgkin lymphoma?
A
Disseminate widely and in an unpredictable fashion early on
7
Q
Small cell lymphoma involves what cell type?
A
B lymphocytes
8
Q
B lymphocytes in small cell lymphoma can be described as ____?
A
Smal and well-differentiated
9
Q
CD19/20, CD23, and CD5
A
CLL/SLL
10
Q
Transformation of CLL/SLL to diffuse large B cell lymphoma is known as ____?
A
Richter syndrome
11
Q
What is the most common form of indolent NHL?
A
Follicular lymphoma
12
Q
t(14;18) is strongly associated with what lymphoma?
A
Follicular lymphoma
13
Q
Overexpression of what gene is seen in follicular lymphoma? What is the result?
A
Overexpression of BCL2, antagonizes apoptosis
14
Q
CD19, BCL2, CD20, CD10
A
Follicular lymphoma and diffuse large B cell lymphoma
15
Q
Centrocytes (small cleaved cells) and centroblasts are seen in histo samples of what lymphoma?
A
Follicular lymphoma
16
Q
What lymphoma is associated with paratrabecular lymphoid aggregates in bone marrow?
A
Follicular lymphoma
17
Q
What is the median survival of follicular lymphoma?
A
7-9 years
18
Q
What is true about aggressive therapy in follicular lmyphoma?
A
Does not improve with aggressive therapy
19
Q
What is the median survival of follicular lmyphoma that transforms into DLBCL?
A
Less than 1 year
20
Q
What is the most common form of NHL?
A
Diffuse large B-cell lymphoma
21
Q
Overexpression of BCL6 is associated with what type of lymphoma?
A
Diffuse large B-cell lymphoma
22
Q
DLBCL tumors with BCL2 rearrangements usually lack ____ rearrangements
A
BCL6
23
Q
CD19, CD20, CD10, BCL6, surface Ig
A
DLBCL
24
Q
What subtype of DLBCL occurs in setting of T-cell immunodeficiency?
A
Immunodeficiency-associated large B-cell lymphoma
25
Neoplastic B cells in immunodeficiency-associated alrge B-cell lymphoma are usually infected with ____
EBV
26
What DLBCL subtype includes malignant pleural or ascitic effusion, mostly in patients with advanced HIV infection?
Primary effusion lymphoma
27
Tumor cells from primary effusion lymphoma are infected with ____
HHV-8
28
Waldeyer ring, oropharyngeal lymphoid tissue including tonisls and adenoids are commonly involved in what lymphoma?
DLBCL
29
BLBCL with ____ translocations have worse prognosis
MYC
30
African, sporadic, and subset in HIV patients are subsets of what lymphoma?
Burkitt lymphoma
31
Endemic Burkitt lymphoma, AKA
African
32
Nonendemic Burkitt lymphoma, AKA
Sporadic
33
All forms of Burkitt lymphoma are associated with translocations of ____ gene on chromosome ____
MYC, chromosome 8
34
What lymphoma is one of the fastest-growing human tumors?
Burkitt lymphoma
35
Burkitt lymphoma MYC translocation partner is usually the IGH locus ____
t(18;14)
36
Essentially all endemic Burkitt lymphomas are latently infected with ____
EBV
37
What lymphoma is associated with a "starry sky" pattern; high mitotic index and numerous apoptotic cells?
Burkitt lymphoma
38
**Surface IgM**, CD19, CD20, CD10, and BCL6
Burkitt lymphoma
39
What Burkitt lymphoma presents as a mass involving the mandible?
Endemic Burkitt lymphoma
40
What Burkitt lymphoma most often appears as a mass involving the ileocecum and peritoneum?
Sporadic Burkitt lymphoma
41
How does Burkitt lymphoma respond to chemotherapy?
Very aggressive but responds well
42
What is the most common B-cell proliferations of neoplastic plasma cells?
Multiple myeloma
43
Free light chains small in size that are excreted in urine are known as ____
Bence Jones proteins
44
Multiple myeloma usually presents as ____
Tumor masses scattered throughout the skeletal system
45
What is Waldenstrom macroglobulinemia?
High levels of IgM lead to symptoms due to hyperviscosity of the blood
46
What type of neoplasm is associated with lytic bone lesions, hypercalcemia, renal failure, and acquired immune abnormalities?
Plasma cell neoplasms
47
Proliferation/survival of myeloma cells in multiple myeloma depends on what cytokine?
IL-6
48
High serum levels of IL-6 in multiple myeloma are associated with ____
Poor prognosis
49
Myeloma-derived MIP1alpha (aka CCL3) causes what in multiple myeloma?
Augments osteoclast formation causing bone destruction
50
What bones are most commonly affected in multiple myeloma?
Vertebral column, ribs, skull, pelvis, femur, clavicle, scapula
51
How does multiple myeloma present on x-ray?
Punched out defects in bone
52
How do flame cells present? What are they associated with?
Fiery red cytoplasm, multiple myeloma
53
How do Mott cells present? What are they associated with?
Multiple grapelike cytoplasmic droplets, multiple myeloma
54
How do Russell bodies present? What are they associated with?
Cytoplasmic globular inclusions, multiple myeloma
55
How do Dutcher bodies present? What are they associated with?
Nuclear globular inclusions, multiple myeloma
56
What is a common cause of death in multiple myeloma?
Infections due to decreased production of normal Igs
57
Excreted light chains toxic to renal tubular epithelial cells, AKA myeloma kidney
Bence Jones proteinuria
58
What causes rouleaux formation of RBCs ("stack of coins") in multiple myeloma?
High level of M protein
59
Plasma cell tumros positive for CD138
Multiple myeloma
60
What is the medial survival of multiple myeloma? How does that chance is multiple bony lesions are present?
4 to 7 years; 6-12 months
61
t(11;14) translocation leading to overexpression of cyclin D1
Mantle cell lymphoma
62
Cyclin D1, CD19, CD20, CD5+, CD23-
Mantle cell lymphoma
63
Where are extranodal tumors in marginal zone lymphoma intially recognized?
Mucosal site, mucosa-associated lymphoid tumors (MALTomas)
64
Marginal zone lymphoma often arises in tissues involved by ____
Chronic inflammatory disorders of autoimmune or infectious etiology
65
How can marginal zone lymphoma be treated?
If inciting agent is eradicated
66
Marginal zone lymphoma involves translocations that up-regulate expression anf function of BCL10 or MALT1, activating ____
NF-kB
67
What neoplasm of CD4+ T calls occurs only in adults infected by HTLV-1?
Adult T-cell leukemia/lymphoma
68
Cloverleaf or flower cells are seen in ____?
Adult T-cell leukemia/lymphoma
69
Mycosis fungoides/Sezary syndrome are different manifestations of of a tumor of ____ that involve ____
CD4+ helper T-cells that involve the skin
70
Histo findings of epidermis and dermis with a cerebriform appearance due to marked infolding of the nuclear membrane are seen in ____?
Mycosis fungiodes/Sezary syndrome
71
What is specific about Sezary syndrome?
Variant with generalized exfoliative erythroderma
72
Cutaneous leukocyte antigen, CCR4, and CCR10
Mycosis fungiodes/Sezary syndrome
73
Rearrangements in ____ gene on chromosome ____ is associated with anaplastic large-cell lymphoma
ALK gene on chromosome 2p23
74
What signaling pathways are triggered in anaplastic large-cell lymphoma?
RAS and JAK/STAT signaling pathways
75
Horseshoe-shaped nuclei and voluminous cytoplasm are hallmark cells of ____?
Anaplastic large-cell lymphoma
76
T-cell lymphomas with ALK rearrangements tend to occur in what age group?
Children or young adults, good prognosis
77
ALK negative anaplastic large cell lymphoma tend to occur in what age group?
Older adults, worse prognosis
78
Reed-Sternberg cells are associated with ____
Hodgkin lymphoma
79
What are the four classic forms of Hodgkin lymphoma?
Nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte depletion
80
What differentiates lymphocytes predominance subtype from classic types?
RS cells have B-cell immunophenotype different from classic types
81
Despute B-cell origin, these cells do not express msot B-cell specific genes
Reed-Sternberg cells
82
Activation of what transcription factor is common in classic Hodgkin lymphoma?
NF-kB
83
What role do R-S cells have in Hodgkin lymphoma?
Secretion of cytokines, chemokines, and other factors results in accummulation of reactive cells in tissue
84
# What cell?
Two nuclear lobes, large inclusion-like nucleoli, and abundant cytoplasm
Diagnostic Reed-Sternberg cell
85
# What cell?
Single nucleus with large inclusion-like nucleous
Mononuclear variant Reed-Sternberg cell
86
# What cell?
Folded or multilobulated nucleus, abundant pale cytoplasm, lies within open surface
Lacunar variant Reed-Sternberg cell
87
What variant of RS cells are seen in nodular sclerosis subtype?
Lacunar variant
88
# What cell?
Multiple infolded nucleur membranes, polyploid nuclei, small nuclei, fine chromatin, and moderate to abundant pale cytoplasm are present
Lymphohistiocytic variant Reed-Sternberg cells
89
What variant of RS cells are present in lymphocyte predominance subtype?
Lymphohistiocytic variant
90
What is the most common subtype of HL?
Nodular sclerosis HL
91
Nodular sclerosis causes deposition of ____ that divide lymph nodes into circumscribed nodules
Collagen in bands
92
PAX5+, CD15 and CD30+, negative for other B-cell markers, T-cell markers, and CD45
Classic HL subtypes
93
What is the prognosis of nodular sclerosis HL?
Excellent prognosis
94
HL subtype: diagnostic R-S cells and mononuclear variants usually plentiful
Mixed cellularity HL
95
R-S cells in mixed cellularity HL are commonly infected with ____
EBV
96
What distinguishes lymphocyte-rich HL from lymphocyte predominance type?
Lymphocyte rich HL involves presence of **frequent mononuclear variants and diagnostic R-S cells** with "classic" immunophenotypic profile
97
What is the prognosis of lymphocyte-Rich HL?
Good to excellent
98
What percent of lymphocyte-rich HL is associated with EBV?
40%
99
What is the least common form of HL?
Lymphocyte-depletion HL
100
What percent of lymphocyte-depletion HL involves RS cells infected with EBV?
Over 90%
101
What HL predominantly occurs in older adults, HIV+ patients, and nonindustrialized countries?
Lymphocyte-depletion
102
What is the prognosis of lymphocyte-depletion HL?
Less favorable than other subtypes
103
Multilobed nuclei, "popcorn cells" are common is what HL subtype?
Nodular lymphocyte precominance HL
104
CD20+, BCL6+, CD15-, CD30-
Nodular lymphocyte predominance HL
105
Nodular sclerosis or lymphocyte predominance HL are more likely to be found in what stages?
Stage I or II
106
Patients with fever, night sweats, and weight loss are more likely to have what HL subtypes?
Mixed-cellularity or lymphocyte depletion subtypes
107
What is the classic spread pattern of HL?
Nodal disease --> splenic disease --> hepatic disease --> marrow and tissue involvement
108
What is the most important prognostic variable in HL?
Tumor stage
