Haematology 9: Lymphoma 2

Question 1

Why is doing Hepatitis B serology important if you suspect a patient has Lymphoma ?

Answer 1

Treatment for the lymphoma will likely deplete B cells and this can cause reactivation of hepatitis B and cause fulminant liver failure

Question 2

Which protein is over-expressed in Follicular lymphoma and can be stained for in germinal centres ?

Answer 2

Bcl2

Question 3

Which NHL is associated with chronic antigen stimulation e.g by viruses ?

Answer 3

Marginal zone lymphomas

Question 4

Give 4 examples of Marginal zone lymphomas and the organism/disease that is associated with it ?

Answer 4

Parotid lymphoma- Sjögren’s syndrome
Gastric MALToma - H.pylori
Thyroid lymphoma - Hashimoto’s thyroiditis
Lacrimal gland lymphoma - Psittaci infection

Question 5

A patient presents with dyspepsia and epigastric pain. Urea breath testing shows likely H.Pylori infection. Which lymphoma is this patient at risk of ?

Answer 5

Gastric MALToma (marginal zone lymphoma -NHL)

Question 6

Which T cell marker is expressed in CLL that would not be expressed in normal B cells ?

Answer 6

CD5

Question 7

Which CD marker is associated with poor prognosis in CLL ?

Answer 7

CD38

Question 8

Which carries a better prognosis for CLL ?

A) IgH mutated
B) IgH unmutated

Answer 8

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

Question 9

Which Chromosomal abnormality has the words prognosis in CLL ?

Answer 9

17p- (TP53)

This deletion causes deletion of the P53 tumour suppressor gene

Question 10

What is a Richter transformation ?

Answer 10

The transformation of CLL into a more aggressive lymphoma, most commonly diffuse large B cell lymphoma

Question 11

In Richter’s transformation CLL most commonly transforms into ………

Answer 11

Diffuse large B cell lymphoma

Question 12

Why do CLL patients require irradiated blood products ?

Answer 12

They are at risk of transfusion associated graft versus host disease

Question 13

What is the 1st line treatment of CLL ?

Answer 13

FCR- Fludarabine, cyclophosphamide and Rituximab

Question 14

name the characteristic 1-2 week cyclical fever seen in lymphomas ?

Answer 14

Pel Ebstein fever

Question 15

What is the treatment for Hodgkin’s lymphoma

Answer 15

ABVD

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

Question 16

Which lymphoma can cause hypercalcaemia and has a clover/flower shaped nucleus?

Answer 16

ATLL

Question 17

Which leukaemia is tartrate resistant acid phosphatase positive ?

Answer 17

Hairy cell leukaemia

Question 18

in which type of lymphoma are reed-sternberg cells seen

Answer 18

classical hodgkin lymphoma or NLPHL

Question 19

subtypes of NHL

Answer 19

B cell - precursor B / mature B cell

T/NK cell - precursor T/ mature T and NK

Question 20

what is the fastest growing human cancer

Answer 20

Burkitt’s lymphoma (form of NHL)

Question 21

3 presentations of NHL

Answer 21

painless lymphadenopathy
compression symptoms
B symptoms

Question 22

two most common types of NHL

Answer 22

diffuse large B cell lymphoma

follicular lymphoma

Question 23

what is the relationship between the grade of a lymphoma and its curability

Answer 23

more aggressive = more curable

Question 24

features and treatment of DLBCL

Answer 24

diffuse large B cell lymphoma 
aggressive 
tx = R-CHOP 
Rituximab 
Cyclophosphamide
Doxorubicin 
Vincristine 
Prednisolone 

50% cure rate

Question 25

features and treatment for follicular NHL

Answer 25

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

Question 26

features of marginal zone lymphomas

Answer 26

``` 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 ```

Question 27

describe MALT lymphomagenesis

Answer 27

lymphocytes respond to H Pylori infection overproliferate cancer-associated changes --> gastric maltoma

Question 28

how is gastric maltoma treated

Answer 28

omeprep/ clarith/ amox repeat breath test 2 months repeat endoscopy every 6 months if fails then chemo

Question 29

features of EATL

Answer 29

``` 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

Question 30

features of chronic lymphocytic leukaemia

Answer 30

proliferation of mature B cells most common leuk lab findings: lymphocytes, smear cells, NN anaemia, thrombocytopenia, BM lymphocytic replacement

Question 31

normal mature B cells express

Answer 31

CD19 positive | CD5 negative

Question 32

what does the presence of CD5 on B cells make you think of

Answer 32

CLL

Question 33

describe treatment for CLL

Answer 33

supportive - vaccination, anti-infective prophylaxis (acyclovir, IVIG) young patients - allogenic SC transplantation chemo - Obinutuzumab + chlorambucil, rituximab - bendamustine, FCR

Question 34

how can high-risk CLL be managed

Answer 34

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