Non-Hodgkin's Lymphoma

Question 1

What is the difference between HL and Non-HL

Answer 1

HL in limited to the lymphatic system
Non-HL includes both Lymphatic and immune systems but still 80% are in Lymph nodes

Question 2

Whatre the 2 main RF of Non-HL?

Answer 2

Infections (viral-mostly or bacterial)
Immunosuppression

Question 3

What infections (both viral and bacterial) are RF/causative agents for Non-HL

Answer 3

Recall: HL is EBV (only classical)

Question 4

H.Pylori is the only bacterial RF for Non-HL, what lymphoma is it associated with?

EBV is a common pathogen that can cause both HL and Non-HL. For Non-HL, what is the associated lymphoma?

Are these high or low grade

Answer 4

MALToma (gastric lymphoma) - Low grade - B cell

Burkitt’s lymphoma - High grade - B cell

Question 5

This is a very distinct rash. What pathogen causes this?

What lymphoma is associated with this pathogen?

Is it low or high grade? B or T cell?

What other diseases does this pathogen cause?

Answer 5

Primary effusion lymphoma - High grade - B cell

Kaposi’s sarcoma - vascular tumour
Castleman’s disease - lymphoproliferative disorder

Question 6

HIV is a nasty disease, so bad that it causes 2 types of lymphomas What are they and are they high or low grade? Are they B or T cell?

What specific HIV is a RF for lymphoma? 1 or 2?

Answer 6

HIV 1
High grade B-cell lymphoma
Primary CNS lymphoma - High grade

both B cells

Question 7

Hepatitis C is one of the chronic infections leading to cirrhosis but luckily, it does not cause a high grade lymphoma. What lymphoma is it a/w and is it B or T cell?

Give 1 other low grade

Answer 7

Marginal zone Lymphoma B cell

Mantle cell lymphoma

Question 8

What are the 2 most common B cell Non-HLs?

What is the grade and morphology? (only for most common)

What is the genetic abnormality associated with this?

What is the oncogene a/w this

Answer 8

It is characterized by large mass localised lesions

Question 9

HTLV-1 is an infection ive never heard of but it is one that causes lymphoma. The nice thing about it is that it is the only one with a T in its name and also the only one that causes a T-cell lymphoma. What is the T-cell lymphoma that it causes?

What is a T cell lymphoma caused by Coeliac disease?

State if they are high or low grade

Now youre set for T-cell Non-HL. There is a cutaneous one => if lymphoma + skin stuff it is cutaneous lymphoma of T helper cells - This one is high grade

Answer 9

HTLV-1 It causes Adult-T cell Lymphoma

Coeliac disease - Enteropathy associated intestinal T-cell lymphoma

both are high grade

Question 10

In order to give differentials, you need to know some types of non-hodgkin’s lymphoma. I hate to do this but you gotta figure it out. Here, let me help you. So the main RF is infections, and each infection has its own tumour. Connect the infections to the tumours.

Answer 10

Question 11

The incidence of Non-HL has doubled in the last 50 years with median age of diagnosis being 10 years increasing with age.
In adults:
What is the most common type (B cell or T cell)
Most common grade (Low vs high)

Answer 11

B cell (80%) > T cell (20%) (Hodgkin’s only B cell, 100%)

Typically low grade as they are clinically indolent (in paeds it is high grade and clinically aggressive)

Question 12

How many cells are involved in HL? How about Non-HL?

I mean the number of different cells involved and not the literal number of cells in total.

Answer 12

single cell in HL
Numerous in Non-HL

Question 13

What is meant by leukemic phase of non-HL?

Answer 13

The leukemic phase in non-HL means that malignant lymphocytes are present and detectable in the blood which is not a feature of HL.

Anyways if it is detectable in the blood,

Question 14

State if each of the following is more likely to indicate HL or Non-HL
Contiguous spread
Large number of tumour cells
CD45
CD15
Involvement of Waldeyer’s Ring
Involvement of peripheral blood
Cutaneous involvement
Extranodal involvement

Answer 14

All Non-HL except CD 15, CD 30 which are Classical HL (nodular HL is CD 20 and 45)

Question 15

Compare HL to Non-HL

Discuss the following if stuck:
Basic pathophysiology and growth pattern
Mode of Spread
Peripheral blood involvement
Number of Tumour cells

Answer 15

Both involve lymphocytes
In HL a single cell proliferates uncontrollably but in Non-HL, they are multi-centric and diffuse in their growth pattern

Contiguous spread in HL but not in Non-HL

In contrast to HL, Non-HL disseminates early and may involve the peripheral blood like leukaemia. This is called the leukemic phase

Question 16

Can lymphomas affect the BM?

Answer 16

Yes, however they must start in the lymphatic system and spread to the BM as opposed to leukemias which start in the BM and spread elsewhere

Question 17

The rate of growth (low vs high grade) dictates treatment and prognosis. What % of low grade non-HL transform into high grade?

Answer 17

30%

Question 18

Are B symptoms common in Non-HL?

Answer 18

They are common in high grade non-HL (e.g. in paeds) => it is not a common finding in adults.

Important note:
B symptoms is considered a poor prognostic factor only for HL and not Non-HL. A way to remember that is that the classification in Non-HL does not include B symptoms like the Ann Arbor classification of HL

Question 19

What are the symptoms of Non-HL?

Answer 19

Question 20

What are the CDs (target antigen) in non-HL for
B cells
T cells
Myeloid (extra)

Answer 20

B-cell = CD19,20 +/-45 (in Classical HL it was CD 15 and 30 and in NHL it was CD20 and 45)
T-cell = CD2,3,7
Myeloid = CD 13,14,33

Question 21

Non-HL involves Triple assessment of the affected tissue. What tests does this entail?

Answer 21

Microscopic assessment
Immuno-phenotype assessment
FISH Genetic Testing

Question 22

Microscopic assessment is part of the Triple assessment for Non-HL.
What does it entail, and what are you looking for? (3)

Answer 22

Whole LN excisional biopsy (not FNA!!!) for histology assessing grade and pattern (follicular vs diffuse)

+ Blood film from peripheral blood to assess for atypical cells

+ BM biopsy for assessing invasion

Question 23

Immuno-phenotype assessment is part of the Triple assessment for Non-HL. What does it entail and what are you looking for? (2)

Answer 23

Immunohistochemistry to determine if B or T cell lineage
B-cell = CD19,20 +/-45 (in Classical HL it was CD 15 and 30 and in NHL it was CD20 and 45)
T-cell = CD2,3,7
Myeloid = CD 13,14,33

Flow cytology allows quantification of cells and subtypes

Just for understanding sake, it is also taken from blood here. In leukemia it was from BM in acute and blood in chronic

Question 24

Go through the diagnostic investigations for Non-HL

Answer 24

1) Microscopic assessment
Whole LN excisional biopsy (not FNA!!!) for histology assessing grade and pattern (follicular vs diffuse)

+ Blood film from peripheral blood to assess for atypical cells

+ BM biopsy for assessing invasion

Immuno-phenotype assessment
Immunohistochemistry to determine if B or T cell lineage
B-cell = CD19,20 +/-45 (in Classical HL it was CD 15 and 30 and in NHL it was CD20 and 45)
T-cell = CD2,3,7
Myeloid = CD 13,14,33

Flow cytology allows quantification of cells and subtypes

FISH Genetic Testing

Question 25

The Ann Arbor Staging System is used for Hodgkin’s lymphoma. What is used for Non-HL

How does it differ from Ann Arbor

Answer 25

The Lugano Classification is used
it is derived form the Ann Arbor and also uses the PET CT FDG except
No “A and B” suffix as B symptoms are not a prognostic factor in Non-HL
Based on number of tumour sites and their location rather than just above and below diaphragm

Question 26

T or F: When discussing the International Prognostic Index Score used for HL and Non-HL, the higher the score, the worse the prognosis

Answer 26

Yes, true