haematology - lymphoma Flashcards
define lymphoma
neoplastic tumour of lymphoid tissue found in
- lymph nodes (often)
- other lymphoid tissue (sometimes)
- anywhere (rarely)
Hodgkin’s lymphoma
20% of lymphomas
M>F, 20-29 and >60yrs
spreads contiguously to adjacent lymph nodes
Hodgkin’s lymphoma is associated with
EBV
clinical presentation of Hodgkin’s lymphoma
asymmetrical painless lymphadenopathy
+/- obstructive/mass effect symptoms
FLAWS
mediastinal/cervical nodes
pain in affected nodes after alcohol
investigations for Hodgkin’s lymphoma
CT/PET
LN/BM biopsy (cells stain with CD15 and CD30)
blood film (Reed Sternberg cells)
Reed Sternberg cells
seen in Hodgkin’s lymphoma
bi-nucleate/multinucleate owl eyed mature lymphocytes
Staging system for Hodgkin’s Lymphoma
Ann Arbor
1: 1 LN region
2: 2+ LN regions on same side of diaphragm
3: 2+ LN regions on opposite sides of diaphragm
4: extra nodal sites
A = no constitutional symptoms
B = constitutional symptoms
treatment overview for Hodgkin’s lymphoma
combination chemotherapy
radiotherapy
intensive chemo and autologous SCT
combination chemotherapy for Hodgkin’s lymphoma
used in most cases
ABVD - adriamycin, bleomycin, vinblastine, and dacarbazine
stage 1/2 = 2-4 cycles
stage 3/4 = 6-8 cycles
radiotherapy for Hodgkin’s lymphoma
used in conjunction with chemo in bulky areas or limited disease
caution of radiotherapy for Hodgkin’s lymphoma
high risk of breast cancer in women
intensive chemotherapy for Hodgkin’s lymphoma and autologous SCT
for relapsed patients
prognosis of Hodgkin’s lymphoma
excellent especially if young and intensively treated
Where are stem cells harvested from for a transplant
peripheral blood
BM
umbilical cord blood
Collecting stem cells from peripheral blood requires
stimulation by G-CSF
SCTs
enables high dose chemo/radiotherapy to eradicate malignant cells at the cost of BM ablation
autologous SCTs
patient’s own SCs harvested, frozen, and reintroduced
autologous SCTs used in
multiple myeloma
lymphoma
benefits of autologous SCTs
no graft vs leukaemia effect
no GVHD
lower risk of infection
allogenic SCTs
HLA-matched donor SCs harvested and introduced to colonise ‘empty’ BM
risks associated with allogenic SCTs
GVHD
opportunistic infections
infertility
secondary malignancies
Non-Hodgkin’s Lymphoma
80% of lymphomas
classified according to:
- mature/immature
- histology
- B/T cell lineage
high grade non-Hodgkin’s lymphomas
very aggressive: Burkitt’s
aggressive: Diffuse large B cell, Mantle cell
low grade non-Hodgkin’s lymphomas
indolent - follicular, marginal zone, small lymphocytic
B cell lymphomas
Burkitt’s
Diffuse large B cell (DLBC)
Mantle cell lymphoma
follicular
MALT
T cell lymphomas
anapaestic large cell lymphoma
peripheral T cell lymphoma
adult T cell leukaemia/lymphoma
enteropathy associated T cell lymphoma (EATL)
cutaneous T cell lymphoma
Burkitt’s lymphoma
aggressive
fast growing
rapidly responsive to treatment
Burkitt’s lymphoma histology
‘starry sky’ appearance
chromosomal abnormality in Burkitt’s lymphoma
t(8; 14) translocation
c-myc oncogene overexpression
types of Burkitt’s lymphoma
endemic
sporadic
immunodeficient
endemic Burkitt’s lymphoma
EBV associated
most common malignancy in equatorial Africa
characteristic features of endemic Burkitt’s lymphoma
jaw involvement
abdominal masses
sporadic Burkitt’s lymphoma
EBV associated found outside Africa
immunodeficient Burkitt’s lymphoma
non-EBV associated
HIV/post-transplant patients
treatment of Burkitt’s lymphoma
chemotherapy (rituximab) and leukaemia protocol
action of rituximab
anti CD20 (found on B cells)
Diffuse large B cell lymphoma (DLBC)
aggressive
Richter’s transformation
DLBCL is most commonly seen among
middle aged and elderly
Richter’s transformation
other lymphomas occur secondary to DLBCL
histology of DLBCL
sheets of large lymphoid cells
treatment of DLBCL
R-CHOP
auto-SCT for relapse
Mantle cell lymphoma
aggressive
disseminated at presentation
Mantle cell lymphoma seen in
middle aged
more commonly men
chromosomal abnormality in mantle cell lymphoma
t(11; 14) translocation
cyclin D1 deregulation
histology of mantle cell lymphoma
angular/clefted nuclei
treatment of mantle cell lymphoma
R-CHOP
auto-SCT for relapse
follicular lymphoma
indolant and mostly curable
chromosomal abnormality in follicular lymphoma
t(14; 18) translocation
histology of follicular lymphoma
follicular pattern
nodular appearance
treatment of follicular lymphoma
watch and wait
rituximab/obinutuzumab + CVP
MALT lymphoma
marginal zone NHL
seen in middle aged
aetiology of MALT lymphomas
chronic antigen stimulation
- H. pylori (gastric MALT lymphoma)
- Sjogren’s syndrome (parotid MALT lymphoma)
treatment of MALT lymphomas
remove antigen stimulus
H. pylori triple therapy
chemotherapy
t(8; 14) translocation
c-myc oncogene overexpression in Burkitt’s lymphoma
t(11, 14) translocation
cyclin D1 deregulation in mantle cell lymphoma
t(14; 18) translocation
follicular lymphoma
c-myc oncogene overexpression
t(8; 14) translocation
Burkitt’s lymphoma
cyclin D1 deregulation
t(11, 14) translocation
mantle cell lymphoma
follicular lymphoma chromosomal mutation
t(14; 18) translocation
R-CHOP chemotherapy
rituximab
cyclophosphamide
doxorubicin hydrochloride
vincristine (oncovin)
prednisolone
CVP chemotherapy
cyclophosphamide
vincristine sulfate
prednisolone
anaplastic large cell lymphoma
aggressive
children and young adults
large ‘epithelioid’ lymphocytes
t(2; 5) translocation
alk-1 protein expression
peripheral T cell lymphoma
aggressive
middle aged and elderly
large T cells
adult T cell leukaemia/lymphoma
aggressive
seen more commonly in Carribean and Japanse
HTLV-1
HTLV-1
human T-lymphotropic virus type 1
oncogenic human retrovirus causing ATL
EATL
enteropathy associated T cell lymphoma
associated with longstanding coeliac disease
Cutaneous T cell lymphoma
associated with mycosis fungicides
