Haematology 9: Lymphoma 2 Flashcards
Why is doing Hepatitis B serology important if you suspect a patient has Lymphoma ?
Treatment for the lymphoma will likely deplete B cells and this can cause reactivation of hepatitis B and cause fulminant liver failure
Which protein is over-expressed in Follicular lymphoma and can be stained for in germinal centres ?
Bcl2
Which NHL is associated with chronic antigen stimulation e.g by viruses ?
Marginal zone lymphomas
Give 4 examples of Marginal zone lymphomas and the organism/disease that is associated with it ?
Parotid lymphoma- Sjögren’s syndrome
Gastric MALToma - H.pylori
Thyroid lymphoma - Hashimoto’s thyroiditis
Lacrimal gland lymphoma - Psittaci infection
A patient presents with dyspepsia and epigastric pain. Urea breath testing shows likely H.Pylori infection. Which lymphoma is this patient at risk of ?
Gastric MALToma (marginal zone lymphoma -NHL)
Which T cell marker is expressed in CLL that would not be expressed in normal B cells ?
CD5
Which CD marker is associated with poor prognosis in CLL ?
CD38
Which carries a better prognosis for CLL ?
A) IgH mutated
B) IgH unmutated
A) IgH mutated
Mutated = arising from post-germinal centre Unmutated = arising from pre-germinal gentre Normally B cells only have mutated heavy chains (IgH) after they undergo somatic hypermutation (VDJ recombination) at germinal centres. Mutated heavy chains mean that the B lymphocyte has been selected for its affinity for a particular antigen. In CLL patients 50% have unmutated heavy chains and this is a much worse prognosis
Which Chromosomal abnormality has the words prognosis in CLL ?
17p- (TP53)
This deletion causes deletion of the P53 tumour suppressor gene
What is a Richter transformation ?
The transformation of CLL into a more aggressive lymphoma, most commonly diffuse large B cell lymphoma
In Richter’s transformation CLL most commonly transforms into ………
Diffuse large B cell lymphoma
Why do CLL patients require irradiated blood products ?
They are at risk of transfusion associated graft versus host disease
What is the 1st line treatment of CLL ?
FCR- Fludarabine, cyclophosphamide and Rituximab
name the characteristic 1-2 week cyclical fever seen in lymphomas ?
Pel Ebstein fever
What is the treatment for Hodgkin’s lymphoma
ABVD
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
Which lymphoma can cause hypercalcaemia and has a clover/flower shaped nucleus?
ATLL
Which leukaemia is tartrate resistant acid phosphatase positive ?
Hairy cell leukaemia
in which type of lymphoma are reed-sternberg cells seen
classical hodgkin lymphoma or NLPHL
subtypes of NHL
B cell - precursor B / mature B cell
T/NK cell - precursor T/ mature T and NK
what is the fastest growing human cancer
Burkitt’s lymphoma (form of NHL)
3 presentations of NHL
painless lymphadenopathy
compression symptoms
B symptoms
two most common types of NHL
diffuse large B cell lymphoma
follicular lymphoma
what is the relationship between the grade of a lymphoma and its curability
more aggressive = more curable
features and treatment of DLBCL
diffuse large B cell lymphoma aggressive tx = R-CHOP Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
50% cure rate
features and treatment for follicular NHL
indolent lymphoma
associated with t(14;18) which results in over-expression of BCL2 (anti-apoptosis protein)
incurable
Tx = watch and wait
combination immunotherapy R-CVP
Rituximab - Cyslophosphamide/ Vincristine/Prednisolone
features of marginal zone lymphomas
involves extranodal lymphoid tissue chronic antigen stimulation median age 55-60 Sjogren, H Pylori... commonly arise in stomach present with dyspepsia or epigastric pain B symptoms uncommon
describe MALT lymphomagenesis
lymphocytes respond to H Pylori infection
overproliferate
cancer-associated changes
–> gastric maltoma
how is gastric maltoma treated
omeprep/ clarith/ amox
repeat breath test 2 months
repeat endoscopy every 6 months
if fails then chemo
features of EATL
enteropathy-associated T cell lymphoma Coeliac pt mature T cells small intestines aggressive chronic antigenic stimulation (gluten/gliadin)
presentation:
abdo pain, malab, obst, perf, systemic symptoms, responds poorly to chemo, fatal
prevent by adherence to strict gluten-free diet
features of chronic lymphocytic leukaemia
proliferation of mature B cells
most common leuk
lab findings: lymphocytes, smear cells, NN anaemia, thrombocytopenia, BM lymphocytic replacement
normal mature B cells express
CD19 positive
CD5 negative
what does the presence of CD5 on B cells make you think of
CLL
describe treatment for CLL
supportive - vaccination, anti-infective prophylaxis (acyclovir, IVIG)
young patients - allogenic SC transplantation
chemo - Obinutuzumab + chlorambucil, rituximab - bendamustine, FCR
how can high-risk CLL be managed
high risk = TP53/17p deletion
refractory disease or early relapse
new agents = ibrutinib (bruton TKI)
venetoclax (anti-Bcl2 oral agent)
CAR-T - chimeric antigen receptor T cells