Introduction To Lymphomas And Myelomas

Question 1

Define lymphoma

Answer 1

cancer of white blood cells (lymphoctes)

- affects mature blood cells mostly B and T lymphocytes

Question 2

What genetic sturcure is associated to lymphoma?

Answer 2

Many specific genetic mutations and chromosomal translocations

Question 3

What is the main functions of lymphatic system? (4)

Answer 3

1) blood filtration / purification
2) removal of excess fluids from tissues
3) absorption and transport of lipids
4) immune system activation

Question 4

What are primary lymph nodes? + examples

Answer 4

Sites where stem cells divide and become imunocompetent

- bone marrow and thymus

Question 5

What happens in seconary lymph nodes + examples

Answer 5

Sites where most of immune responses occurs

- spleen, peyer’s patches, lymph nodes, tonsils

Question 6

How do lymphomas develop stage wise?

Answer 6

Lymphocytes affected in diff maturation states due to genetic alteration

Uncontrolled division of the lymphocytes

Orga size increases e.g. splenomegaly(spleen), adenopathy (lymph node)

Spreads to other tissues through lymphatic system

Infiltrate into bone marrow - detectable in blood and/or other organs

Question 7

What is the classification of lymphomas?

Answer 7

Non hodgkin lymphoma - most common - separates into either diffuse or follicular
Hodgkin lymphoma

Question 8

Presentation of lymphoma

Answer 8

Enlarged lymph nodes, normally painless
Excessive night sweats
Unintended weightloss
Itching
Constant tiredness

Question 9

How do you diagnose lymphoma?

Answer 9

Lymph node biopsy - flow cytometry, FISH, NGS and immunophenotyping

Question 10

How do you diagnose stage of lymphoma after diagnosing lymphoma?

Answer 10

PET scan (positon emission tomography)

Question 11

What are the 4 stages of lymphoma?

Answer 11

Stage 1 - localise - single lymph node / organ
Stage 2 - 2 or more lymph nodes but on same side of diaphragm
Stage 3 - 2 or more lymph nodes but on diff sides of diaphragm
Stage 4 - widespread, multiple organs with or without lymph node involvement (liver and bone marrow)

Question 12

What causes lymphoma?

Answer 12

Multifactorial - malfunction to body’s immune system and exposure to certain infections

usually B cell develops a mutation in its DNA

Question 13

Risk factors of hodgkin lymphoma

Answer 13

Epstein barr virus
Familt history
HIV/AIDS

Question 14

How does Hodkin lymphoma present?

Answer 14

Non painful enlarged lymph nodes

Question 15

How do you diagnose hodkin lymphma?

Answer 15

Excisional lymph node biopsy

- look for reed - sternberg cells (hodgkin cell only in hodgkin lymphoma)

Question 16

What are reed sternberg cells?

Answer 16

Hodgkin cells

Multinucleated red stem cells in lymph nodes - only in Hodgkin not non hodgkin lymphoma

Question 17

What is the treatment for hodgkin lymphoma?

Answer 17

Chemotherapy and sometimes radiotherpay too.

Stem cell transplant

Question 18

What is non-hodgkin lymphoma?

Answer 18

all lymphomas apart from hodgkin lymphomas

Question 19

How does non hodgkin lymphoma present?

Answer 19

Enlarged lymph nodes + general symtoms including night swears, fever and weight loss)

Question 20

What is the cause of non hodgkin lymphoma?

Answer 20

Chromosomal translocation

esp involving Ig heavy chain or light chain loci on chromosome 14
- each Ig gene has powerful tissue specific enhancer

Question 21

What are risk factors of non hodgkin lymphomas?

Answer 21

virus infections (EBV) driven lymphomas in immunosuppressed pateints e.g  Burkitt's lymphoma
Human T cell leukemia virus in adult T cell lymphomas

Question 22

What is the normal role of an enhancer ig Ig genes? and how does it contribute to lymphomas?

Answer 22

Activate promoter of rearrnaged V segment

If rearrnaged infront of oncogene during chromosomal translocation, enhancer promotes expression of oncogene protein = non hodgkin lymphoma

Question 23

What chromosomal translocation happens in non hodkin lymphoma?

Answer 23

Translocation between chromosome 14 and 18 bringing the enhancer infront of BCL-2 gene causing overexpression of BCL-2

Question 24

What is BCL-2 gene?

Answer 24

encoded for BCL-2 (an apoptosis inhibtor) enahnced = excess cel proliferation due to low apoptosis

Question 25

Which chromosome translocations in in Burkitt’s lymphoma (type of nonhodkin lymphoma)

Answer 25

Chromsomes 8 MYC gene (oncogene)
Chromsome 14 IgH gene - enhancer

fuses causing constant oncogene gain of function

Question 26

How do virus infections e.g. EBV act as risk factor for non hodgkin lymphomas?

Answer 26

Viral oncogene carried in viruses (LMP-1 oncogene in EBV)

Question 27

What is EBV and how does it contribute to non hodgkin lymphomas?

Answer 27

Epstein Barr virus - almost everyone carries latently but in highly immunosuppressed indivuduals, endogenous latent EBV transform B cells and no longer elimiate T cytoxic T cells
- results in high grade lymphoma

Question 28

What are the classifications of non hodgkin lymphomas?

Answer 28

Low grade

High grade

Question 29

Comapre high grade and low grade lymphoma

Question 30

How do you diagose non hodgkin lymphomas?

Question 31

How do you treat non hodgkin lymphomas

Question 32

What are multiple myelomas?

Question 33

How does multiple myelomas present?

Question 34

What are the causes/risk factors of multiple myelomas?

Question 35

What is a paraprotein? and how is it charcaterised

Question 36

What 3 features of myeloma give rise to different clinical features

Question 37

v

Question 38

How do you diagnose mutliple myelomas?

Question 39

What is the treatment for multiple myelomas?