Intro to Lymphoma

Question 1

3 places where
1. B cells
2. NK cells
are often found

Answer 1

1. Lymph nodes, spleen, MALT

2. Skin, liver, spleen, GI tract

Question 2

What is a lymphoid neoplasm?

Answer 2

Lymphoproliferation due to uncontrolled accumulation of lymphocytes
Malignant accumulation of neoplastic lymphocytes

Question 3

Lymphoma definition

Answer 3

Collection of malignant lymphocytes that form a mass

Question 4

What does the
1. t(14;18)
2. t(8;14)
translocation lead to?

Answer 4

1. Loss of apoptosis

2. Uncontrolled cellular proliferation

Question 5

t(14;18)

Answer 5

Chromosome 14 has an Ig heavy chain gene that is constitutively expressed
Chromosome 18 has the BCL-2 gene that is anti-apoptosis
When they are fused there is constitutive expression of the BCL2 gene and over expression of anti-apoptosis protein BCL2

Question 6

t(8;14)

Answer 6

Chromosome 14 has an Ig heavy chain gene that is constitutively expressed
Chromosome 8 has the MYC gene that promotes cellular proliferation
When they are fused there is constitutive expression of the MYC gene and over expression of cell proliferation promoter MYC protein

Question 7

5 oncogenic viruses

Answer 7

EBV
HIV
Hep C
Human T-lymphotropic virus 1 (HTLV-1)
Human herpes virus 8 (HHV-8)

Question 8

How does Epstein Barr Virus lead to lymphoma?

Answer 8

Infects B cells and incorporates DNA into host genome

These cells can get genetic transformations that lead to lymphoma

Question 9

4 ways to get genetic transformation that can lead to lymphoma

Answer 9

Random genetic alteration
Oncogenic virus
Antigen over stimulation
Immunosuppression

Question 10

2 common causes of antigen over-stimulation

Answer 10

Microorganisms

Autoimmune diseases

Question 11

Historial convention

1. Hodgkin lymphoma
2. Non-hodgkin lymphoma

Answer 11

1. Tumor consists mostly of reactive inflammatory cells with only rare Reed-Sternberg cell lymphocytes
2. All the cells in the tumor are malignant lymphocytes

Question 12

Indolent/low grade lymphoma

Answer 12

Tumour grows slowly over the course of years
Lymphocyte accumulation due to defective apoptosis
Malignant lymphocytes are usually small
Treatment often not required at time of diagnosis
Incurable

Question 13

Aggressive/high grade lymphoma

Answer 13

Tumour grows rapidly over the course of hours, days or weeks
Lymphocyte accumulation due to uncontrolled cellular proliferation
Malignant lymphocytes are usually large
Treatment required at the time of diagnosis
Potentially curable

Question 14

Lymphoma is more common in…. (age group, sex, race, cell type, location)

Answer 14

Adults and elderly (30+)
Males
Caucasians
B cell origin
Lymph nodes

Question 15

Low grade lymphoma on history and physical exam

Answer 15

Slow growing painless mass
Sometimes multiple masses at multiple sites
Constitutional symptoms usually ABSENT
Appears well
+/- hepatosplenomegaly
If bone marrow is involved, can see pallor, bruising, signs of infection

Question 16

High grade lymphoma on history and physical exam

Answer 16

Rapidly growing mass
Often localized
Constitutional B symptoms (weight loss, fever, night sweats)
Appear unwell
Febrile
Non-tender lymphadenopathy
\+/- hepatosplenomegaly
Abdominal mass
If bone marrow is involved, can see pallor, bruising, signs of infection

Question 17

3 types of biopsy you can do to diagnose lymphoma

Answer 17

Fine needle aspiration
Needle core biopsy (sample of node is taken)
Excisional biopsy (remove entire lymph node)

Question 18

Treatment of lymphoma depends on

Answer 18

Diagnosis and classification of the lymphoma

Question 19

Low stage vs high stage disease

Answer 19

Low (stage 1/2): disease localized to one or a few lymph node groups or organs, good prognosis
High (stage 3/4): disease spread throughout the body, involves lymph nodes and extra nodal sites, poor prognosis

Question 20

4 ways lymphoma can be treated

Answer 20

Conservative management (watch and wait)
Chemo (mainstay)
Immunotherapy
Radiation

Question 21

4 main lymphomas

Answer 21

Classical hodgkin lymphoma
Follicular lymphoma
Diffuse large B-cell lymphoma
Burkitt lymphoma

Question 22

Classical Hodgkin Lymphoma (what is it, clinical features, treatment)

Answer 22

Reed-Sternberg cell in a background of mixed inflammatory cells
Clinical features: lymphadenopathy, B symptoms common, pruritis, lymph node pain after alcohol consumption
Treat with chemo

Question 23

Follicular lymphoma

Answer 23

Middle age to elderly adults, rare in children
Clinical features: predominantly involves lymph nodes, B symptoms less common
Associated with t(14;18)
Low grade, small B cell lymphoma, follicular growth pattern
Incurable
Conservative management or immunochemo

Question 24

Diffuse Large B cell lymphoma

Answer 24

Most common lymphoma, most frequently in elderly, males
Clinical features: affects lymph nodes and extra-nodal sites equally, rapidly enlarging mass at single or multiple sites, often otherwise asymptomatic
High grade B cell lymphoma (large cells), diffuse growth pattern
Treat with immunochemo
Aggressive
Can be curable

Question 25

Burkitt Lymphoma

Answer 25

Most common non-Hodgkin lymphoma in a pediatric population
Can be endemic, sporadic or immunodeficiency associated
Features: rapidly growing tumour
High grade B cell lymphoma (large cells), diffuse growth pattern, mitotic figures and apoptotic bodies are abundant
Treat with immunochemo