Haematology 8: Lymphoma 1 Flashcards
Which is more common Hodgkin lymphoma or NHL ?
NHL (80%)
B cell = more common than T cell
Where does VDJ recombination occur ?
Bone marrow
Where does class switching occur ?
Germinal centres
Which lymphoma is associated with chronic H.pylori infection?
Gastric MALT lymphoma
Which lymphoma is associated with Coeliac disease ?
EATL - enteropathy-associated T cell Lymphoma
Which virus causes adult T cell lymphoma ?
HTLV1
Which CD marker is a marker of Germinal centre B cells ?
CD10
What does Follicular lymphoma look like on Histology ?
Follicular pattern- lots of follicles in lymph tissue
BCL2 possitive
Which 2 Lymphomas is associated with EBV infection ?
Burkitt’s lymphoma
Hodgkin’s lymphoma
Which lymphoma has a starry-sky appearance
Burkitt’s lymphoma
Which Lymphoma is particularly prevelant in the Caribbean and japan ?
Adult T cell Lymphoma
Which cell allows differentiation between Hodgkin’s and NHL lymphoma ?
Reed-sternberg cells (seen in Hodgkin’s)
looks like Owl eyes
Describe the staging of Hodgkin Lymphoma from 1-4 A/B
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
Name the staging system in Hodgkins lymphoma ?
Ann-Arbor
where are lymphomas usually found
lymph nodes, bone marrow. blood
lymphoid organs - spleen or GALT
skin
rarely anywhere - CNS, oscular, testes, breasts
describe the process of immunoglobulin and T cell receptor gene recombination
bone marrow - VDJ recombination of germ line genes germinal centre - class switching (DNA alterations) and somatic hypermutation (insertion of nucleotide point mutations)
list malignant genes which may be expressed if you translocate an oncogene downstream of the promoter
Bcl2
Bcl6
Myc
cyclinD1
aetiology of lymphoma
majority have no identifiable RF
- constant antigenic stimulation
- infection
- loss of T cell function
what lymphomas result from H. Pylori infection
gastric MALT
marginal zone NHL of the stomach
what lymphomas result from sjogrens syndrome
marginal zone NHL of parotic lymphoma
what lymphomas result from coeliac disease
small bowel T cell lymphoma, EATL
what virus can cause adult T cell leukaemia lymphoma
HTLV1
how can EBV cause lymphomas
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)
name examples of :
- generative lymphoid tissue
- reactive lymphoid tissue
- acquire lymphoid tissue
generative = BM and thymus reactive = LN and spleen acquired = extra-nodal lymphoid tissue (skin, stomach, lung)
roles of T cells
express surface T cell receptor
regulation of B cells and macrophages
cytotoxic function
what do normal lymph nodes look like
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)
what is the main B cell marer
CD20
what is the main T cell marker
CD3 (CD5)
how can lymphoma be classified
HODGKIN:
- classical
- lymphocyte prediminant
NHL:
- B cell - precursor B, peripheral B (high/low grade)
- T cell - precursor T cell, peripheral T cell
diagnostic tools in lymphoma
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
what are common low grade B cell lymphomas
follociular lymphoma
small lymphocytic lymphoma/ chronic lymphocytic
manginal zone
mantle zone
what are common high grade lymphomas
diffuse large B cell lymphoma
what is a common intermediate grade lymphoma
Burkitt’s lymphoma
features of follicular lymphoma
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
features of small lymphocytic /chronic lymphocytic leaimaemia
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)
features of marginal zone lymphoma/ MALT lymphoma
arise mainly at extranodal sites
arises after chronic antigenic stimulation
arise from post-germinal centre memory B cells
features of mantle cell lymphoma
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
features of Burkitts lymphoma
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
diffuse large b cell lymphoma
middle aged and elderly
lymphadenopathy
large lyphoid cells
germinal centre phenotype phenotype = good prognosis
p53 positive and high proliferation fraction - poor prognosis
features of T cell lymphomas
middle aged and elderly lymphadenopathy and extranodal sites large T lymphocytes reactive cell population (esp eosinophils) aggressive
features of anaplastic large cell lymphoma
children and young adults lymphadenopathy large epitheloid lymphocytes 2;5 translocation alk-1 protein expression = better prognosis aggressive
differences between NH and H lymphoma
H is more localised - only one nodal site
H spreads contiguously
NHL involves multiple lymph node sites and spreads discontinuously
features classical hodgkin lymphoma
young and middle aged single group of LN EBV associations histopath: sclerosis, Reed sternbeg/ hodgkin cells, eosinophils diagnostic markers = CD30, CD15
nodular lymphocyte predominant hodgkin lymphoma
isolated lymphadenopathy no association with Ebv arise from germinal B cells negative for CD30 and CD15 positive for CD20
features of hodgkin lymphoma
bimodal age 20-29, 60+ painless enlarged LN nodes can cause obstructive signs + symptoms B symptoms alcohol induced pain
how is hodgkin lymphoma staged
1 - one group of LN
2 - >1 group of LN same side of diaphragm
3 - nodes above and below diaphragm
4 - extra nodal spread
how is hodgkin lymphoma treated
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
prognosis for hodgkin lymphoma
80% in stage 1/2 are cured
50% of stage 4 are cured
80% are long-term survivors
