B3.041 - Pre-work Intro to Lymphoid Neoplasms Flashcards
(114 cards)
1
Q
Where can lymphoid neoplasms be derived from
A
Precursor cells (B or T ALL/LBL) Mature B cells Mature T/NK cells
2
Q
What is are the 3 possible presentations of lymphoid neoplasms
A
primarily in: Bone marrow and blood Lymph nodes extranodal tissues
3
Q
Classification of lymphoid neoplasms are based on what
A
cell of origin Developmental stage of transfromed cell clinical, immunophenotypic, genetic and molecular findngs
4
Q
Classification of lymphoid neoplasms doe snot distinguis what
A
if its primarily blood presentation or lymph presentation
5
Q
What is the primary involvement of leukemia’s
A
Bone marrow or blood
6
Q
What is the origin of leukemia’s
A
Myeloid or lymphoid
7
Q
What might leukemia’s secondarily involve
A
Lymph nodes and solid tissues
8
Q
What is the tissue involvement of lymphomas
A
Lymph node or solid tissue
9
Q
What is the origin of lymphomas
A
Lymphoid
10
Q
What might lymphomas secondarily involve
A
Bone marrow and blood
11
Q
Chronic lymphocytic leukemia aka
A
Small lymphocytic lymphoma
12
Q
Acute lymphoblastic leukemia aka
A
Lymphoblastic lymphoma
13
Q
Acute lymphoblastic leukemia aka
A
Lymphoblastic lymphoma
14
Q
What does acute mean with leukemia’s
A
Primarily immature cells (blasts) Untreated has a rapidly progressive track and is fatal in weeks to months
15
Q
What does chronic mean in regards to leukemia’s
A
Primarily mature or maturing cells Untreated slowly progressive and fatal in months to years
16
Q
About 80% of all lymphomas are derived from what
A
B Cells
17
Q
How much of lymphomas are derived from T or NK cells
A
20%
18
Q
What is the most common extranodal mature T neoplasm located in
A
Skin
19
Q
What is the most common skin lymphoma
A
Mycosis fungoides
20
Q
What are the markers of mature B cell neoplasms
A
CD19, CD20
21
Q
What do mature T cell neoplasms express
A
CD2 CD3 CD5 CD7 CD4 CD8 (variable)
22
Q
Any given B cell neoplasm will express what
A
The same Igs, meaning they’re monoclonal
23
Q
What does monoclonal mean
A
All these cells with have a kappa light chain or a lambda light chain but not both
24
Q
What does monoclonal surface Ig mean clinically
A
You can distinguish between monoclonal neoplasms from non neoplastic responses
25
Which CDs do not help to distinguish clonality
CD4 and CD8
26
Describe the genetics of mature T cell neoplasms
Monoclonal rearranged T cell receptor genes
27
Describe the genetics of mature B cell neoplasms
Monoclonal rearranged immunoglobulin genes
28
In mature B neoplasms there are often translocations involving what
Chromosome 14
29
Why do translocations to chromosome 14 often result in cancer?
Its the IgH gene locus which is always on
30
Describe lymph node architecture
Diffuse Modular Follicular
31
Describe small lymphoid cell size features
Tend to be more indolent Immunophenotype and genetics re critical for diagnosis and classification
32
Describe characteristics of medium-large lymphoid
Tend to be more aggressive Morphology supplemented by immunophenotype and genetics for diagnosis and classification
33
What are the most common B cell neoplasms
CLL/SLL
34
What are the clinical features of CLL and SLL
Older adults, most common leukemia in US variable anemia/thrombocytopenia Lymphadenopathy Immunologic abnormalities
35
what is the morphology of CLL
small mature lymphocytes in blood, bone marrow
36
What is the morphology of SLL
diffuse infiltrate of small lymphocytes in lymph nodes
37
What is the characteristic immunophenotype of CLL/SLL
CD19+, CD20+, CD5+ CD10- slg+(clonal)
38
What is characteristic of mutated CLL/SLL
Somatic hypermutation in IgVh (\>2% variance from germline sequence 40-50% of cases)
39
what other lymphoma shares these immunophenotypes
mantle cell lymphoma
40
what is the favorable type of CLL/SLL
Mutated
41
what is unmutated CLL/SLL
no somatic hypermutation in IgVh
42
what are other genetic abnormalities with CLL/SLL
Deletion 13q, deletion 11q, deletion 17p, trisomy 12
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which other genetic abnormality in CLL/SLL is considered favorable
deletion 13q
44
what is the median survival of CLL/SLL
6 years
45
why is the median survival of CLL/SLL misleading
its 3 y for unmutated and over 7 for mutated
46
what is hairy cell leukemia clinically seen in
middle aged older adults
47
what are clinical features of hairy cell leukemia
splenomegaly hepatomegaly pancytopenia infections
48
What is the morphology of hairy cell leukemia
lymphocytes with villous projections
49
what is the immnophenotype of hair cell leukemia
CD19+, CD20+, CD11c+ slg+ (Clonal) CD25+ CD103+ annexin A1 CD10-, CD5-
50
what are the genes associated with hairy cell leukemia
BRAF mutation
51
which immunophenotypes are specific for hairy cell leukemia
CD103 and Annexin A1
52
What are clinical feature of B cell neoplasm follicular lymphomas
Middle aged and older adults, common lymphadenopathy hepatosplenomegaly
53
What is the morphology of follicular lymphoma
nodular infiltrate of small cleaved lymphocytes in lymph nodes diminished mantle zone; absent tingible body macrophages
54
what is the immunophenotype of follicular lymphoma
CD19+, CD20+, CD10+, CD5-, slg+ (clonal)
55
what is a gene mutation associated with follicular lymphoma
t(14;18) - overexpression of Bcl-2 protein prevents apoptosis
56
what are clinical features of B cell neoplasm mantle cell lymphoma
middle aged and older adults, uncommon lymphadenopathy, hepatosplenomegaly, often adnvanced stage
57
what is the morphology of mantle cell lymphoma
nodular or diffuse infiltrate of small lymphocytes in lymph nodes
58
what is the immunophenotype of mantle cell lymphoma
CD19+, CD20+, CD10-, CD5+, slg+ (clonal) Cyclne D1+
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what is a gene mutation associated with mantle cell lymphoma
t(11;14) - overexpression of cyclin D1
60
what is the course and prognosis of mantle cell lymphoma
3-4 y poor prognosis
61
what is a main difference between CLL/SLL and mantle cell lymphoma given they share many of the same immunophenotypes
CLL/SLL is very indolent Mantle cell is very aggressive
62
how do you distinguish between CLL/SLL and mantle cell
look for the genetic mutation of t(11;14)
63
what are the clinical feature of B cell neoplasms marginal zone lymphoma
Extranodal - GI, lung, skin,eye, head and neck Association with immune reactions and chronic infection
64
what is the morphology of marginal zone lymphoma
infiltrate of the lymphoid cells into epithelium and expanded marginal zone
65
what is the immunophenotype of marginal zone lymphoma
CD19+, CD20+, CD10-, CD5-, slg+ (clonal)
66
early on in the disease if its being caused by chronic infection how can you treat it
antibacterials
67
what are clinical features of B cell neoplasm diffuse large B cell lymphoma
middle aged and older adults lymphadenopathy hepatosplenomegaly frequently extranodal
68
what is the morphology of diffuse large B cell lymphoma
diffuse infiltrate of large lymphocytes in lymph nodes
69
what is the immunophenotype of diffuse large B cell lymphoma
CD19+, CD20+, CD10+/-, CD5-, slg+ (Clonal) some types associated with EBV or HHV8
70
what are genetic mutations associated with diffuse large B cell lymphoma
Variable, may be positive for MYC translocation
71
what is significant about diffuse large B cell lymphomas with a MYC transolaction
They are more aggressive
72
what are burkitt lymphoma clinical features
Rapidly progressive tumor mass extremely high growth fraction tumor lysis syndrome common when treatment initiated
73
where is burkitt lymphoma endemic to and who does it affect
equatorial africa children mostly mostly associated with EBV
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what form of burkitt lymphoma is common in US, who does it affect and what is it associated with
sporadic form, children and adults, EBV
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what other disease is burkitt lymphoma associated with
HIV
76
nearly all of the cancer cells in burkitt lymphoma are constantly dividing, what does this mean clinically
it grows very fast and is very responsive to treatment tumor lysis syndrome is an issue
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what do you give to patients to prevent tumor lysis syndrome
give lots of fluids and kidney protection
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which from of burkitt lymphoma does the best in respose to treatment
endemic form
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which type of burkitt lymphoma does the worst in response to treatment
HIV associated
80
what is the morphology of burkitt lymphoma
diffuse infiltrate of medium sized lymphocytes in lymph nodes high mitotic rate; tingible body macrophages ("Starry sky")
81
what is the immunophenotype of burkitt lymphoma
CD19+, CD20+, CD10+, CD5-, slg+ (clonal), TdT-
82
what are genetic mutations relevant to burkitt lymphoma
t(8;14) [variants t(2;8) or t(8;22)] - MYC oncogene translocated to IgH locus and constitutively expressed
83
if you see starry sky what should you think
burkitt lymphoma
84
how do you distinguish between burkitt lymphoma and follicular lymphoma
the morphology is different
85
what does TdT- tell you for diagnosis
its burkitts lymphoma and not ALL
86
is the MYC mutation unique to burkitt lymphoma
no
87
which type of lymphoma has an almost 100% mitotic fraction
Burkitt
88
what is mycosis fungoides/sezary syndrome
T cell neoplasm of the skin that can go into the blood
89
what are clinical feature of mycosis fungoides
older adults cutaneous patch, plaque and nodule stages lymphadenopathy unusual
90
what is the morphology of mycosis fungoides
MF - dermal iniltrate of atypical lymphocytes with epidermotropism and pautrier microabscesses SS - circulating lymphoid cells with cerebrioform nuclei and eryhroderma
91
what is the immunophenotype of mycosis fungoides
CD4+
92
what is the course and prognosis of mycosis fungoides
indolent, chronic course with 90% 5 year survivial
93
what are clinical features of adult T cell leukemia/lymphoma
endemic to japan, caribbena, west africa middle aged to older adults caused by HTLV-I generalized lymphadenopathy, skin lesions, hepatosplenomegaly, lymphocytosis, hypercalcemia
94
what infection causes adult .T cell leukemia
HTLV-1
95
what are clinical features of T cell neoplasm anaplastic large cell lymphoma
lymphadenopathy
96
what is the immunophenotype of anaplastic large cell lymphoma
CD30+
97
what are the genetics of anaplastic large cell lymphoma
t(2;5) - rearrangement of ALK
98
what indicates a poor prognosis for anaplastic large cell lymphoma
ALK-
99
what are clinical feature of peripheral T cell lymphoma
lymphadenopathy fever, pruritus, weight loss eosinphilia
100
what is course and prognosis of peripheral T cell lymphoma
poor prognosis with 20-30% 5 year survival
101
what are clinical features of hodgkin lymphoma
bimodal age distribution (15-35 y and \>50) most common malignancy in 10-30 age group in US lymphadenopathy, splenomegaly constitutional B symtoms Immune dysfunciton possible relation to EBV infection
102
what is the morphology of classical hodgkin lymphoma
Reed-Sternberg cells in a polymorphous cellular backgroudn
103
What is the morphology of nodular lymphocyte predominant hodgkin lymphoma
No Reed-sternberg cells; large B cells in a background of small lymphocytes
104
what are constitutional B symptoms
fever night sweats weight loss
105
what are characteristics specific to hodgkin lymphoma
Hodkin - Heterogenous cell population Reed sternberg cells Primary extranodal presentation rare Often localized to a single nodal group spread along contiguous nodal groups Waldeyer ring and mesenteric nodes rarely involved young adults an dedlerly Good prognosis
106
what are characteristics specific to non hodgkin lymphoma
homogenous cell population no reed sternberg cells Primary extranodal presentation common Frequently involve multiple nodal groups Spred to any site in an unpredictable manner Waldeyer ring and mesenteric nodes commonly involved Any age variable prognosis
107
what is the immunophenotype of HL
Reed sternberg cells CD15+, CD30+. CD20 +/-, neg for most other B and T cell markers
108
what is a genetic mutation associated with HL
mutated germinal center B cells that escape apoptosis
109
what is course and prognosis of HL
excellent at early stage Risk of second malignancy in long term survivors
110
Describe CD20 status on all B cell neoplasms with small lymphocytes
+ or all
111
Describe CD5 status for all B cell neoplasms with small lymphocytes
+ for CLL/SLL, Mantle cell - for Hairy cell, MALT, follicular
112
Describe the CD10 status of all B cell neoplasms with small lymphocytes
+ for Follicular, - for the rest
113
Non neoplastic proliferations of lymphocytes
viral infections Acute bacterial infections chronic bacterial infections atypical lymphocytes
114
non neoplastic lymphadenopathy
follicular hyperplasia due to infections Paracortical hyperplasia due to viral infections/drugs Sinus histiocytosis - lymph nodes draining infection
