Non-Hodgkin's Lymphoma

Question 1

What is NHL?

Answer 1

Malignant tumours of lymphoid tissue that do not contain Reed-Sternberg cells

Question 2

What percentage are B cell in origin and what percentage T cell?

Answer 2

70% are B cell in origin

30% T cell

Question 3

Is it the most common haematopoietic malignancy?

Answer 3

Yes

Question 4

What does the incidence increase with?

Answer 4

Age

Question 5

What is the median age of presentation?

Answer 5

55-75

Question 6

Do all the conditions involve lymph nodes?

Answer 6

No - extranodal generating lymphoma including mucosa-associated lymphoid tissue e.g gastric MALT
Spleen
BM
Thymus 
Oropharynx

Question 7

Is there a slight male or female predominance?

Answer 7

Male

Question 8

What risk factors are there?

Answer 8

Elderly, Caucasians
History of viral infection specifically EBV
FH
Certain chemical agents - pesticides, solvents
History of chemotherapy or radiotherapy
Immunodeficiency- transplant, HIV, DM
Autoimmune disorders- SLE, Sjogrens, coeliac, RA..

Question 9

How can NHL be classified?

Answer 9

Low grade - B cell or T cell

High grade - B cell or T cell

Question 10

Is HL or NHL more common?

Answer 10

NHL

Question 11

What symptoms and signs are associated?

Answer 11

Superficial, painless, rubbery lymphadenopathy
B symptoms - weight loss, fever, night sweats
Constitutional symptoms - lethargy, pruritis
Extranodal involvement:
GI - abdominal pain, dyspepsia, constipation or diarrhoea
Oropharynx (Waldeyer’s ring) - dysphagia, obstructed breathing
BM - fatigue, bleeding and bruising, recurrent infections, bone pain
Lungs - SOB, cough
Skin - red patches or scaly lumps under skin
CNS - headaches, seizures, weakness/numbness

Hepatosplenomegaly

Question 12

Do B symptoms typically occur earlier in HL or NHL?

Answer 12

Earlier in HL

B symptoms in NHL suggest disseminated disease

Question 13

Is extra nodal disease more common in NHL or HL?

Answer 13

Much more common in NHL

Question 14

What lymph nodes are typically affected?

Answer 14

Cervical
Axillary
Inguinal

Question 15

What is the diagnostic investigation of choice?

Answer 15

Excisional node biopsy NOT FNA

Question 16

What imaging is needed?

Answer 16

CTCAP to assess staging

Question 17

What tests should be done?

Answer 17

FBC and blood film
U&E
Uric acid - some aggressive NHLs can cause high levels
LFTs - if liver mets expected
ESR - prognostic indicator
LDH - a marker for cell turnover, useful as a prognostic indicator

Bone marrow aspiration and trephine are routine
HIV test - this is a RF for NHL
LP if CNS involvement

Question 18

What staging system is most commonly used?

Answer 18

Ann Arbor

Question 19

Describe low grade lymphomas

Answer 19

Indolent, often incurable and widely disseminated
Follow a replacing and remitting course
Median survival = 10 years
Accounts for 45% of cases

Question 20

How can B and T tumours be distinguished?

Answer 20

Immunophenotyping- dome on blood, marrow or nodal material

Question 21

What types of low grade lymphoma are there?

Answer 21

Follicular lymphoma
Marginal zone / MALT
Lymphocytic lymphoma - closely related to CLL
Hairy cell leukaemia

Question 22

Describe high grade lymphoma

Answer 22

More aggressive
More likely to be cured
Often rapidly enlarging lymphadenopathy with systemic symptoms
80% will initially respond to treatment

Question 23

What types of aggressive NHLs are there?

Answer 23

Diffuse large B cell = most common
Burkitt’s lymphoma
Lymphoblastic lymphoma
Mantle cell

Question 24

Describe Burkitt’s lymphoma

Answer 24

Commonly seen in West Africa
Childhood disease 
Highly aggressive 
Associated with jaw lymphadenopathy 
Associated with EBV

Question 25

What is the link between Burkitt’s lymphoma and malaria?

Answer 25

Malaria thought to reduce resistance to EBV, allowing the virus to take hold

Question 26

How is low grade type treated?

Answer 26

Depends on sub type present If symptomless - likely no treatment given and monitored RT may be curative if localised disease Chlorambucil (chemotherapy) can be used if diffuse Remisssion may be maintained with alpha interferon or rituximab

Question 27

How is high grade lymphoma treated?

Answer 27

``` Chemotherapy regime: R-CHOP Rituximab Cyclophosphamide Hydroxydaunorubicin Vincristine (Oncovin) Prednisolone ```

Question 28

What can be given to help neutropenia?

Answer 28

GCSF

Question 29

What suggests poor prognosis?

Answer 29

Over 60 Bulky disease e.g abdominal mass > 10cm Raised LDH Disseminated disease

Question 30

Rituximab kills what cells?

Answer 30

CD20+

Question 31

Some lymphomas can produce IgM leading to what?

Answer 31

Hyperviscosity syndrome - IgM is the biggest gamma globulin | Autoimmune cytopenias e.g AIHA

Question 32

Some lymphomas produce very few gammaglobulins. What does this lead to?

Answer 32

Infections