MH2

Question 1

What is NHL?

Answer 1

Non Hodgkin’s Lymphoma

Question 2

What causes NHL?

Answer 2

Viruses
Environmental Exposure
Chronic Antigen Exposure

Question 3

What is the difference between Lymphoma and Leukaemia?

Answer 3

Lymphoma is a tissue disease

Leukaemia is disease of the blood

Question 4

How frequent is NHL?

Answer 4

NHL is the sixth most frequent malignancy in the UK

Question 5

What is staging?

Answer 5

Cancer staging is the process of determining the extent to which a cancer has developed by spreading.

Question 6

Describe stage I lymphoma

Answer 6

Lymphoma:

In one group of lymph nodes or in just one organ or area of the body outside the lymphatic system (extranodal lymphoma)

Question 7

Describe stage II lymphoma

Answer 7

Lymphoma:

In 2 or more groups of lymph nodes on the same side of your diaphragm, or
In 1 or more groups of lymph nodes and also in one nearby organ or area of body, all on the same side of the diaphragm

Question 8

Describe stage III lymphoma

Answer 8

Lymphoma:

In lymph nodes on both sides of the diaphragm, or
In lymph nodes on both sides of the diaphragm and a nearby organ or area of body is affected

Question 9

Describe stage IV lymphoma

Answer 9

Lymphoma:

Throughout one or more organs that are not part of the lymphatic system, or
In an organ that is not part of the lymphatic system, and it has also spread to organs or lymph nodes far away from the organ, or
In your liver, bone marrow, cerebrospinal fluid (CSF) or lung (unless it has spread to your lung from nearby lymph nodes)

Question 10

What staging investigations can be performed?

Answer 10

Clinical examination
FBC
Liver function
CXR
CT, or PETCT scan
Bone marrow examination

Question 11

What prognostic markers can be used in evaluating NHL prognosis?

Answer 11

ESR
LDH
Anaemia
Comorbidity (performance status)
Age
Stage

Question 12

Give examples of low grade lymphomas

Answer 12

Follicular NHL
Small lymphocytic lymphoma/Chronic lymphocytic leukaemia
Hairy cell leukaemia
Marginal Zone lymphoma
Lymphoplasmacytic Lymphoma
– All behave similarly...

Question 13

Which low grade NHL is most common?

Answer 13

Follicular NHL

Question 14

Which two low grade NHL make up the majority of NHL’s?

Answer 14

Follicular NHL

DLBCL

Question 15

Describe the presenting features of low grade lymphomas

Answer 15

Painless lymphadenopathy
Abdominal mass
B Symptoms
– Weight loss
– Night sweats

Question 16

How is follicular lymphoma characterised?

Answer 16

Follicular lymphoma is characterised by a translocation between chromosome 14 and 18 results in the overexpression of the bcl-2 gene.

As the bcl-2 protein is normally involved in preventing apoptosis, cells with an overexpression of this protein are basically immortal.

Question 17

What are the treatment options for low grade lymphomas?

Answer 17

Aim for long term control
Rare cures in early stage disease (Stage I)
Otherwise incurable even with high dose therapy
• ? May be curable with allogeneic transplant
May transform to aggressive disease
– poor prognosis
Develop resistance to drugs over time
– Successive remissions shorter
• Eventual progression of refractory disease

Question 18

What are the treatment options for Follicular Lymphoma?

Answer 18

‘Watch and Wait’ – no treatment
Chemotherapy plus antibody (anti-CD20)
– Chemo
• Alkylating agents – chlorambucil (gentle)
• Combination chemotherapy – CHOP CVP etc
• Fludarabine combinations
• Bendamustine………..others….
Anti-CD20 maintenance therapy
Autografts/allografts
?small (targeted) molecules – eg BCL2 inhibitor, BTKi…others…..

Question 19

How is Rituximab used in Follicular Lymphoma?

Answer 19

Induction treatment with chemotherapy
– Initial treatment and relapse
Maintenance
– for 2 years after initial treatment or relapse Rituximab monotherapy
– can also be used for patients with relapsed/refractory Stage III/IV disease where other treatment options have been exhausted.

Question 20

Apart from Rituximab, what other antibodies can be used to treat Follicular Lymphoma?

Answer 20

Anti-CD20 as a means of targeting radiation
– 131 I-Tositumomab labelled anti-CD20 – Bexxar
– 90Y-labeled ibritumomab tiuxetan – Zevalin
Later generation anti-CD20
– Better cellular and complement mediated killing
– Trials awaited……

Question 21

What are the presenting features of high grade lymphomas?

Answer 21

Painless peripheral lymphadenopathy
Abdominal mass
B symptoms
– Weight loss
– Night sweats

Question 22

What are the prognostic features of high grade lymphomas?

Answer 22

IPI score (International Prognostic Index)
– Age
– Stage
– LDH
– Performance score (how fit patient is)
– Extranodal sites
Other issues
– CNS involvement – poor prognosis

Question 23

How is treatment for DLBCL approached?

Answer 23

AIM FOR CURE

Cyclical Combination Chemotherapy with Rituximab – 6-8 courses of RCHOP
Dual modality treatment for early stage disease RCHOPx3 + Radiotherapy
Radiotherapy to sites of initial bulk disease
Salvage therapy and autograft if relapse
– Autograft at relapse can be curative still.

Question 24

What are some key issues involved in HGL?

Answer 24

Getting treatment in on time is important
CNS disease
– CNS directed chemotherapy
CNS prophylaxis for patients at high risk of CNS relapse.
– High IPI
– Extranodal disease – eg Breast
– Disease close to CNS

Question 25

Give examples of High Grade NHL

Answer 25

Burkitts Lymphoma

Mantle Cell NHL

Question 26

Describe the features of Burkitts Lymphoma

Answer 26

– Very, very aggressive
– Tumour lysis (rapid cell death) a problem
– Needs intensive chemotherapy with CNS prophylaxis
– Majority cured if survive the first 6 months

Question 27

Describe the features of Mantle Cell Lymphoma

Answer 27

– Variable grade – indolent to blastic
– Difficult to treat
– Poor prognosis - 3-4 yrs

Question 28

What are the presenting features of Hodgkin’s Disease?

Answer 28

Painless lymphadenopathy
– Often cervical/mediastinal
– Contiguous Spread
Systemic symptoms/B Symptoms
– Weight loss/fevers/Night sweats

Question 29

How is HD classified?

Answer 29

Nodular lymphocyte predominant HD – very indolent!
Classical HD
 - Lymphocyte rich
 - Nodular sclerosis
 - Mixed cellularity
 - Lymphocyte depleted

Question 30

What investigations can be performed for HD?

Answer 30

Histology
CT PET scan
BM
ESR/LDH/FBC/Biochemistry
Baseline cardiac and lung function
Follow up CT/PET to assess response after 2 cycles of treatment

Question 31

How is HD treated?

Answer 31

Stage IA & IIA – divided into low and high risk - bulk/age/esr etc
– Abbreviated chemotherapy + IF-RT
• Involved Field – limits radiation to where it is needed
– Higher risk early stage may be treated as later stage
disaese

Stage IB – IVB
– Cyclical combination chemotherapy
     • ABVD – current standard of care
     • Others
– MOPP/COPP
– ChlVPP/PABLOE and other hybrid regimens
– Stanford V
     • BEACOPP/eBEACOPP – more aggressive treatment

Question 32

What is the prognosis for HD?

Answer 32

Most are cured (70%)

Better if
– good risk.
– Young age
– Low bulk – IA
– Good histology?
RATHL trial after 3 yrs
– 95%OS, 82%PFS......
PET scans after two cycles predictive of long-term response

Question 33

What issues relate to long term survival of HD?

Answer 33

Therapy related toxicity a big issue!

Secondary malignancy – cumulative risk > 10%
– NHL/MDS or AML/solid tumours.
– Breast cancer risk high in young women treated with Radiotherapy prior to age 30
Early cardiovascular disease
Infertility
Lung problems
Psychosocial issues.