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IBI Test 3 > B3.041 Prework Intro to Lymphoid Neoplasms > Flashcards

B3.041 Prework Intro to Lymphoid Neoplasms Flashcards

(78 cards)

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1
Q

where can lymphoid neoplasms be derived from?

A

precursor cells (B or T ALL)
mature B cells
mature T/MK cells

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2
Q

where can lymphoid neoplasms present?

A

primarily bone marrow/blood
primarily in lymph nodes
primarily in extranodal tissues

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3
Q

how are lymphoid neoplasms classified

A

cell of origin
development stage of transformed cell (precursor or mature)
clinical, immunophenotypic, genetic, and molecular findings
DOES NOT distinguish primarily blood/BM (leukemia) from primarily lymph node/tissue presentation (lymphoma)

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4
Q

same as chronic lymphocytic leukemia

A

small lymphocytic lymphoma

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5
Q

same as acute lymphoblastic leukemia

A

lymphoblastic lymphoma

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6
Q

what % of all lymphoid neoplasms involve B cells

A

80%

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7
Q

what % of all lymphoid neoplasms involve T/NK cells

A

20%

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8
Q

what type of lymphoma is classified neither as T or B cell?

A

Hodgkin

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9
Q

what are the 5 most common non-Hodgkin lymphomas?

A 
diffuse large B cell lymphoma
follicular lymphoma
MALT lymphoma
mature T cell lymphomas
CLL/SLL
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10
Q

which 3 mature B cell neoplasms occur in the BM/blood

A

CLL/SLL
hairy cell leukemia
Burkitt

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11
Q

which 5 mature B cell neoplasms occur in the lymph nodes

A 
CLL/SLL
follicular lymphoma
diffuse large B cell lymphoma
mantle cell lymphoma
Burkitt
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12
Q

which 2 mature B cell neoplasms occur extranodally

A

diffuse large B cell lymphoma

marginal zone lymphoma

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13
Q

which 2 mature T cell neoplasms occur in the BM/blood

A

adult T cell leukemia/lymphoma

mycosis fungoides/Sezary syndrome

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14
Q

which 3 mature T cell neoplasms occur in the lymph nodes

A

peripheral T cell lymphoma, unspec
anaplastic large cell lymphoma
adult T cell leukemia/lymphoma

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15
Q

which mature T cell neoplasm occurs extranodally

A

mycosis fungoides/ Sezary syndrome

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16
Q

what are some immunophenotypic markers of mature B neoplasms

A

CD19, CD20
monoclonal surface Ig (K or L restricted)
others disease based

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17
Q

what are some immunophenotypic markers of mature T neoplasms

A

CD2, CD3, CD5, CD7 (some may be absent)
CD4 and/or CD8 (variable, do NOT establish clonality)
others diseased based

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18
Q

genetic features of mature B neoplasms

A

monoclonal rearranged Ig genes
translocations involving chromosome 14 (IgH gene locus, constitutively expressed)
disease specific findings

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19
Q

genetic features of mature T neoplasms

A

monoclonal rearranged T-cell receptor genes (a/B or y/d)

disease specific findings

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20
Q

what are 2 different possible lymph node architectures of lymphoid neoplasms?

A

diffuse

nodular/follicular

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21
Q

distinguish between small and medium-large cell lymphoid neoplasms

A

small
-more indolent
-immunophenotype and genetics are critical for diagnosis and classification
medium-large
-tend to be more aggressive
-morphology supplemented by immunophenotype and genetics for diagnosis and classification

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22
Q

clinical features of chronic lymphocytic leukemia/ small lymphocytic lymphoma (CLL/SLL)

A 
older adults
most common leukemia in the US
variable anemia, thrombocytopenia
lymphadenopathy
immunologic abnormalities- autoimmune anemia or thrombocytopenia, monoclonal immunoglobulin (paraprotein)
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23
Q

morphology of CLL/SLL

A

CLL - small mature lymphocytes in blood, BM

SLL- diffuse infiltrates of small lymphocytes in lymph nodes

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24
Q

histologic features of CLL/SLL

A

smudge like cells w no cytoplasm

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25
immunophenotype of CLL/SLL
CD19+ CD20+ CD5+ sIg+ (clonal)
26
genetics of unmutated CLL/SLL (U-CLL/SLL)
no somatic hypermutation in IgV 40-50% of cases unfavorable
27
genetics of mutated CLL/SLL (M-CLL/SLL)
somatic hypermutation in IgV 50-60% of cases favorable
28
course and prognosis of CLL/SLL
``` median survival 6 y -worse for U-CLL/SLL (3) than M-CLL/SLL (7) prolymphocytic transformation - 15-30% -mean survival <2 y Richter transformation - 5-10% -large cell lymphoma -increased lymphadenopathy -mean survival <1 y ```
29
clinical features of hairy cell leukemia
middle aged and older adults rare splenomegaly, hepatomegaly pancytopenia, infections
30
morphology/histological features of hairy cell leukemia
villous projections
31
immunophenotype of hairy cell leukemia
``` CD19+ CD20+ sIg+ (clonal) CD11c+ CD25+ CD103+ ** annexin A1 ** **very specific ```
32
genetics of hairy cell leukemia
BRAF mutation | also seen in other cancers, targeted therapies exist
33
course and prognosis of hairy cell leukemia
indolent
34
clinical features of follicular lymphoma
middle aged and older adults common (2nd most common non Hodgkin) lymphadenopathy hepatosplenomegaly
35
morphology of follicular lymphoma
nodular infiltrate of small cleaved lymphocytes in lymph nodes diminished mantle zone absent tangible body macrophages
36
immunophenotype of follicular lymphoma
CD19+ CD20+ CD10+ sIg+ (clonal)
37
genetics of follicular lymphoma
t(14;18) | overexpression of BCL2 which prevents apoptosis
38
course and prognosis of follicular lymphoma
indolent
39
clinical features of mantle cell lymphoma
middle aged and older adults uncommon lymphadenopathy hepatosplenomegaly (often advanced stages)
40
morphology of mantle cell lymphoma
nodular or diffuse infiltrate of small lymphocytes in lymph nodes
41
immunophenotype of mantle cell lymphoma
``` CD19+ CD20+ CD5+ sIg+ (clonal) above same as CLL/SLL cyclin D1+ ```
42
genetics of mantle cell lymphoma
t(11;14) overexpression of cyclin D1 | involved in cell division (oncogene)
43
course and prognosis of mantle cell lymphoma
poor prognosis median survival 3-4 y unusually aggressive for small cell
44
clinical features of marginal zone lymphoma
extranodal - GI tract, lung, skin, eye, head & neck association with immune reaction and chronic infection (H. pylori) -can sometimes be treated early by treating underlying infection
45
morphology of marginal zone lymphoma
infiltrate of lymphoid cells into epithelium and expanded marginal zone
46
immunophenotype of marginal zone lymphoma
CD19+ CD20+ sIg+ (clonal)
47
course and prognosis of marginal zone lymphoma
indolent
48
clinical features of diffuse large B cell lymphoma
``` middle aged and older adults most common lymphoma lymphadenopathy hepatosplenomegaly frequently extranodal ```
49
morphology of diffuse large B cell lymphoma
diffuse infiltrate of large lymphocytes in lymph nodes
50
immunophenotype of diffuse large B cell lymphoma
``` CD19+ CD20+ CD10+/- sIg+ (clonal) some types associated with EBV or HHV8 ```
51
genetics of diffuse large B cell lymphoma
may be positive for MYC translocation (most aggressive)
52
course and prognosis of diffuse large B cell lymphoma
aggressive
53
clinical features of Burkitt
``` rapidly progressive tumor mass -extremely high growth fraction -tumor lysis syndrome common w treatment -fast, but responsive to treatment endemic form in Africa -primarily kids - >95% associated with EBV -mandible, abdomen sporadic form in US -involved kids and adults -15-20% associated with EBV -abdomen HIV associated form -worst response to therapy -25% associated with EBV ```
54
morphology of Burkitt
diffuse infiltrate of medium sized lymphocytes in lymph nodes high mitotic rate tingible body macrophages (starry sky)
55
immunophenotype of Burkitt
``` CD19+ CD20+ CD10+ sIg+ (clonal) TdT- (can look like ALL, but ALL is TdT+) ```
56
genetics of Burkitt
t(8;14) [variants t(2;8) or t(8;22)] MYC oncogene translocated to IgH locus and constitutively expressed
57
course and prognosis of Burkitt
highly aggressive course but high cure rates (60-90%) w chemotherapy
58
clinical features of mycosis fungoides/sezary syndrome
older adults cutaneous patch, plaque, nodule stages lymphadenopathy unusual
59
morphology of MF/SS
MF- dermal infiltrate of atypical lymphocytes with epidermotropism and Pautrier microabcesses SS- circulating lymphoid cells with cerebriform nuclei and erythroderma
60
immunophenotype of MF/SS
CD4+ T cells
61
course and prognosis of MF/SS
indolent chronic course with 90% 5 year survival SS more aggressive form
62
clinical features of adult T cell leukemia/lymphoma
endemic in Japan, Caribbean, West Africa middle aged to older adults caused nu HTLV-1 infection generalized lymphadenopathy, skin lesion, hepatosplenomegaly, lymphocytosis, and hypercalcemia
63
course and prognosis of adult T cell leukemia/lymphoma
poor prognosis especially in leukemic phase
64
clinical features of anaplastic large cell lymphoma
lymphadenopathy
65
immunphenotype of anaplastic large cell lymphoma
CD30+
66
genetics of anaplastic large cell lymphoma
t(2;5) rearrangement of ALK
67
course and prognosis of anaplastic large cell lymphoma
poor prognosis if ALK-
68
clinical features of peripheral T-cell lymphoma
``` lymphadenopathy fever pruitis weight loss eosinophilia (secretion of IL-5) ```
69
course and prognosis of peripheral T cell lymphoma
poor prognosis with 20-30% 5 year survival rate
70
clinical features of Hodgkin
bimodal age distribution (15-35 and >50) most common malignancy in 10-30 y age group in US lymphadenopathy, splenomegaly constitutional ("B") symptoms- fever, night sweats, weight loss immune dysfunction possible relation to EBV
71
morphology of Hodgkin
classical - Reed-Sternberg cells in a polymorphous cellular background nodular lymphocyte predominance - no Reed-Sternberg cells; large B cells in a background of small lymphocytes
72
describe Reed-Sternberg cells
extremely large 2 nuclei large nucleous with clearing around it
73
what are some features that make Hodgkin unique from non-hodgkin
``` heterogeneous population reed-Sternberg primary extranodal presentation rare often localized to a single nodal group spread along contiguous nodal groups tonsils and adenoids rarely involved young adults and elderly good prognosis ```
74
immunophenotype of Hodgkin
``` reed Sternberg cells CD15+ CD30+ CD20+/- negative for most other T cell markers ```
75
genetics of Hodgkin
mutate germinal center B cells that escape apoptotic death
76
course and prognosis
excelled prognosis in early stage (90% cure rate) | risk of secondary malignancy in long term survivors (due to chemo and radiation)
77
non-neoplastic proliferations of lymphocytes
viral infections acute bacterial infections chronic bacterial infections atypical lymphocytes
78
non-neoplastic lymphadenopathy
follicular hyperplasia- infection, autoimmune disorders, drug reaction paracortical hyperplasia- viral infections, drug reactions sinus histiocytosis- lymph nodes draining infections of cancer

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