Haematology 8: Lymphoma 1 Flashcards

1
Q

Which is more common Hodgkin lymphoma or NHL ?

A

NHL (80%)

B cell = more common than T cell

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2
Q

Where does VDJ recombination occur ?

A

Bone marrow

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3
Q

Where does class switching occur ?

A

Germinal centres

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4
Q

Which lymphoma is associated with chronic H.pylori infection?

A

Gastric MALT lymphoma

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5
Q

Which lymphoma is associated with Coeliac disease ?

A

EATL - enteropathy-associated T cell Lymphoma

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6
Q

Which virus causes adult T cell lymphoma ?

A

HTLV1

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7
Q

Which CD marker is a marker of Germinal centre B cells ?

A

CD10

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8
Q

What does Follicular lymphoma look like on Histology ?

A

Follicular pattern- lots of follicles in lymph tissue

BCL2 possitive

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9
Q

Which 2 Lymphomas is associated with EBV infection ?

A

Burkitt’s lymphoma

Hodgkin’s lymphoma

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10
Q

Which lymphoma has a starry-sky appearance

A

Burkitt’s lymphoma

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11
Q

Which Lymphoma is particularly prevelant in the Caribbean and japan ?

A

Adult T cell Lymphoma

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12
Q

Which cell allows differentiation between Hodgkin’s and NHL lymphoma ?

A

Reed-sternberg cells (seen in Hodgkin’s)

looks like Owl eyes

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13
Q

Describe the staging of Hodgkin Lymphoma from 1-4 A/B

A

Stage 1: one group of nodes
Stage 2: >1 group of nodes same side of diaphragm
Stage 3: nodes above and below the diaphragm
Stage 4: extranodal spread

A: without any B symptoms
B: Fever, Unexplained weightloss of more than 10% in 6 months, night sweats

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14
Q

Name the staging system in Hodgkins lymphoma ?

A

Ann-Arbor

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15
Q

where are lymphomas usually found

A

lymph nodes, bone marrow. blood
lymphoid organs - spleen or GALT
skin
rarely anywhere - CNS, oscular, testes, breasts

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16
Q

describe the process of immunoglobulin and T cell receptor gene recombination

A
bone marrow - VDJ recombination of germ line genes
germinal centre - class switching (DNA alterations) and somatic hypermutation (insertion of nucleotide point mutations)
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17
Q

list malignant genes which may be expressed if you translocate an oncogene downstream of the promoter

A

Bcl2
Bcl6
Myc
cyclinD1

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18
Q

aetiology of lymphoma

A

majority have no identifiable RF

  • constant antigenic stimulation
  • infection
  • loss of T cell function
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19
Q

what lymphomas result from H. Pylori infection

A

gastric MALT

marginal zone NHL of the stomach

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20
Q

what lymphomas result from sjogrens syndrome

A

marginal zone NHL of parotic lymphoma

21
Q

what lymphomas result from coeliac disease

A

small bowel T cell lymphoma, EATL

22
Q

what virus can cause adult T cell leukaemia lymphoma

A

HTLV1

23
Q

how can EBV cause lymphomas

A

when EBV is quiescent, cytotoxic T cells kill EBV antigen expressing B cells
loss of T cell function –> risk of EBV driven lymphomas
T cell function lost due to HIV/organ transplantation immunosuppression (leads to PTLD)

24
Q

name examples of :

  • generative lymphoid tissue
  • reactive lymphoid tissue
  • acquire lymphoid tissue
A
generative = BM and thymus 
reactive = LN and spleen 
acquired = extra-nodal lymphoid tissue (skin, stomach, lung)
25
Q

roles of T cells

A

express surface T cell receptor
regulation of B cells and macrophages
cytotoxic function

26
Q

what do normal lymph nodes look like

A

rounded areas = B cell follicles
between B cell follicles = T cell areas
medulla = central area where mature B cells end up
B cells migrate from the mantle zone inwards to the germinal centre (where they encounter APC and undergo activation and selection)

27
Q

what is the main B cell marer

A

CD20

28
Q

what is the main T cell marker

A

CD3 (CD5)

29
Q

how can lymphoma be classified

A

HODGKIN:
- classical
- lymphocyte prediminant
NHL:
- B cell - precursor B, peripheral B (high/low grade)
- T cell - precursor T cell, peripheral T cell

30
Q

diagnostic tools in lymphoma

A

cytology
histology
- architecture - nodular/diffuse
- cells - small round, small cleaved, large
immunophenotyping
FISH - id chromosomal translocations
PCR - id chromosome translocations, clonal T cell receptor, or Ig gene rearrangement

31
Q

what are common low grade B cell lymphomas

A

follociular lymphoma
small lymphocytic lymphoma/ chronic lymphocytic
manginal zone
mantle zone

32
Q

what are common high grade lymphomas

A

diffuse large B cell lymphoma

33
Q

what is a common intermediate grade lymphoma

A

Burkitt’s lymphoma

34
Q

features of follicular lymphoma

A

presents as lymphadenopathy in the middle-aged/elderly
follicular pattern
germinal cell origin (positive staining for CD10 and bcl-6
14;18 translocation involving bcl2 gene
neoplastic follicles express bcl-2 on immunohistochemistry

35
Q

features of small lymphocytic /chronic lymphocytic leaimaemia

A

presentation = middle aged/elderly
detected in LN/blood
histopath = small lymphocytes. arises from B cells or post-germinal centre memory B cells
CD5 and CD23 positive
multiple genetic abnormalities
indolent, but can transform into a higher grade lymphoma (Richter transformation)

36
Q

features of marginal zone lymphoma/ MALT lymphoma

A

arise mainly at extranodal sites
arises after chronic antigenic stimulation
arise from post-germinal centre memory B cells

37
Q

features of mantle cell lymphoma

A

middle-aged males
affects LN and GI tract
often presents with disseminated disease
histopath - in the mantle zone of the lymph
arise from pre-germinal centre structures
aberrant expression of CD5 and cyclin D1
11;14 transclocation
median survival 3-5 yrs

38
Q

features of Burkitts lymphoma

A
jaw/abdominal mass in children/young adults
associated with EBV 
arises from germinal centre cells 
starry-sky appearance 
c-myc translocation (8;14,2;8 or 8;2)
aggressive disease
39
Q

diffuse large b cell lymphoma

A

middle aged and elderly
lymphadenopathy
large lyphoid cells
germinal centre phenotype phenotype = good prognosis
p53 positive and high proliferation fraction - poor prognosis

40
Q

features of T cell lymphomas

A
middle aged and elderly
lymphadenopathy and extranodal sites
large T lymphocytes 
reactive cell population (esp eosinophils)
aggressive
41
Q

features of anaplastic large cell lymphoma

A
children and young adults 
lymphadenopathy 
large epitheloid lymphocytes
2;5 translocation 
alk-1 protein expression = better prognosis
aggressive
42
Q

differences between NH and H lymphoma

A

H is more localised - only one nodal site
H spreads contiguously
NHL involves multiple lymph node sites and spreads discontinuously

43
Q

features classical hodgkin lymphoma

A
young and middle aged 
single group of LN 
EBV associations 
histopath: sclerosis, Reed sternbeg/ hodgkin cells, eosinophils
diagnostic markers = CD30, CD15
44
Q

nodular lymphocyte predominant hodgkin lymphoma

A
isolated lymphadenopathy 
no association with Ebv 
arise from germinal B cells 
negative for CD30 and CD15 
positive for CD20
45
Q

features of hodgkin lymphoma

A
bimodal age 20-29, 60+ 
painless enlarged LN 
nodes can cause obstructive signs + symptoms 
B symptoms 
alcohol induced pain
46
Q

how is hodgkin lymphoma staged

A

1 - one group of LN
2 - >1 group of LN same side of diaphragm
3 - nodes above and below diaphragm
4 - extra nodal spread

47
Q

how is hodgkin lymphoma treated

A
all should receive chemo 
radiotherapy also given 
combined modality = R+ C 
chemotherapy = ABVD 
Adriamycin 
Bleomycin 
Vincristine 
Decarbazine (DTIC)
4 weekly intervals 
preserves fertility 
long-term consequences = pulmonary fibrosis + cardiomyopathy
48
Q

prognosis for hodgkin lymphoma

A

80% in stage 1/2 are cured
50% of stage 4 are cured
80% are long-term survivors