lymphoma Flashcards
what is lymphoma
Neoplastic tumour of lymphoid tissue
• Often lymph nodes (+ Bone marrow +/- spill out to blood)
• Sometimes other lymphoid tissues – spleen, MALT (mucosal associated lymphoid tissue)
• Rarely, “anywhere” – skin (often T-cell), CNS, testes, breast
what is the epidemiology of hodgkins
- M>F; bimodal age incidence – 20-29 year olds and >60 year olds
- EBV-associated
- Spreads contiguously to adjacent lymph nodes; often involves single LN group
What are the clinical symptoms of hodgkins
• Asymmetrical painless lymphadenopathy +/- obstructive/mass effect symptoms
• “B-symptoms” o Fever >38. Classical Pel-Ebstein fever (cyclical 1-2wk) seen in a minority o Drenching sweats at night
o Weight loss >10% in 6 months unintentional
• Pain in affected nodes after alcohol
• Nodes tend to be mediastinal / cervical but not always
what investigations in hodgkins
- CT/PET. Tissue diagnosis: LN or BM biopsy - cells stain with CD15 & CD30
- Reed-Sternberg cell – bi-nucleate/multinucleate (‘owl eyed’) cell on a background of lymphocytes & reactive cells
- Subtypes: nodular sclerosing (most common), mixed cellularity, lymphocyte rich, lymphocyte depleted, nodular lymphocyte predominant (not classical HL)
ann arbor staging
Stage 1 – one LN region (LN region can include spleen)
Stage 2 – two or more LN regions on the same side of the diaphragm
Stage 3 – two or more LN regions on opposite sides of the diaphragm
Stage 4 – extranodal sites (liver, BM)
what is the treatment for hodgkins
- Combination chemotherapy – o Used in most cases
o ABVD: Adriamycin, bleomycin, vinblastine and dacarbazine o 2-4 cycles in stage 1/2, 6-8 cycles in stage 3/4 - Radiotherapy – o Often used alongside chemo in bulky areas or limited disease– very high risk of breast cancer in women
- Intensive chemo (e.g. BEACOPP) and autologous SCT – o Relapsed patients
what acronym for hodgkins chemo
ABVD
classic histology of burkitts
starry sky
three types of burkits
endemic, sporadic, immunodeficiency
which subtype of burkitts has jaw involvement
endemic
types of non hodgkin lymphoma
High Grade
Very Aggressive – Burkitt’s
Aggressive – Diffuse Large B-Cell, Mantle Cell o Low Grade
Indolent – Follicular, Marginal Zone, Small Lymphocytic
diffuse large b cell
Middle aged and
elderly Aggressive Richter’s transformation
Other lymphomas occur secondary to
DLBCL
what is the histology of diffuse large b cell lymphoma
“Sheets of large lymphoid
cells”
mantel cell lymphomas
Middle-aged, M>F
Aggressive Disseminated at presentation Median survival 3-5 years
t(11;14) translocation Cyclin D1 deregulation
follicular lymphoma
Indolent
Mostly incurable
Median survival 12-15 yrs
t(14:18) translocation
histology of lymphoma follicular
follicular or nodular pattern
malt
Marginal zone NHL
Middle-aged Chronic antigen stimulation:
• H. pylori à gastric MALT lymphoma
• Sjogren’s syndrome à parotid lymphoma
t cell lymphomas
anaplastic large cell lymphoma, peripheral t cell lymphoma, adult t cell lymphoma, enteropathic associated t cell lymphoma, cutaneous t cell lymphoma
which lymphoma is associated with coeliac disease
enteropathy associated t cell lymphoma (EATL)
Which lymphoma is associated with mycosis fungoides
cutaneous t cell lymphoma
anaplastic large cell lymphoma
Children and young adults
Aggressive
Large “epithelioid” lymphocytes t(2;5)
Alk-1 protein expression
what are some pathways to lymphoma
immune system disease (drives disease by constant antigen stimulation e.g. h pylori - MALT), loss of T cell function (permits EBV driven b cell lymphoma)
lymphoma histology
o Generative LR tissue generation/maturation of lymphoid cells
Bone marrow and thymus
o Reactive LR tissue development of immune reaction
Lymph nodes and spleen
o Acquired LR tissue development of local immune reaction
Extra-nodal lymphoid tissue (e.g. skin, stomach, lung)
lymphoma CD markers
o CD19, CD20 = B-cells
o CD3, CD5 = T-cells
hodgkins lymphoma
o S/S: Young and middle-aged, single group of lymph nodes
o Arises from the germinal centre or post-germinal centre cells
o Associated with EBV
o Histopathology
Sclerosis
Mixed cell population with Reed-Sternberg/Hodgkin cells (binucleate ‘owl’s’ eyes)
Lymphoma cells are relatively few in number and tend to be scattered around
Eosinophils
o Prognosis:
Moderately aggressive
Diagnostic markers = CD30, CD15
Hodgkins vs non nodhkins lymphoma
Hodgkins is more localised and spreads contiguosly to adjacent lymph nodes
what are the types of HL
NODULAR SCLEROSING, MIXED CELLULARITY, LYMPHOCYTE RICH
What is the histopathology of hodgkins lymphoma
CD5, CD19 markers,
owl eyes - reed sternberg cells
eosinophils,
What is the features of nodular lymphocyte predominant lymphoma
80% of HL, F > M (20-29yo)
o Neck nodes and a mediastinal mass; may have B symptoms
o Spreads contiguously
o Needs tissue diagnosis
what are the diagnostic markers for hodgkins lymphoma
cd5, cd19
how is hodgkins lymphoma treated
with Combined therapy, chemo first and then radio to eliminate remaining cells
what chemo is used for hodgkins
ABVD - adriamycin, Bleomycin, vinblastine, dacarbazine
what are the features of follicular NHL
It is indolent, i.e.g incurable with a prognosis of 15 years
name some extra nodal marginal zone lymphomas
MALToma-h pylori
parotid lymphoma - psjogrens
lacrimal gland lymphoma - psittaci
thyroid - hashimotos
what are the features of EATL
Very aggresive cancer associated with excessive gliadin exposure in coeliac patients