Non Hodgkin's lymphoma

Question 1

What is a lymphoma?

Answer 1

A type of cancer that develops from lymphocytes, lymphoma can arise in the lymph nodes (nodal) or in other sites (extra nodal)

Question 2

What are the 2 broad subtypes of lymphoma?

Answer 2

• Hodgkins (15%)

* Non-Hodgkin’s

Question 3

What are the categories of Hodgkin’s lymphoma?

Answer 3

• Classical (>90%)

* nodular lymphocyte predominant

Question 4

What are the categories of non-hodgkin lymphoma?

Answer 4

•B cell
•T cell
These categories are both further subdivided into indolent and aggerssive

Question 5

How do patients with lymphoma present?

Answer 5

•Lymphadenopathy - either painless or rubbery
•Splenomegaly
•Extranodal disease e.g. breast, brain or lung
•B symptoms:
- night sweats
- weight loss
- unexplained fever
•Anaemia ( due to bone marrow infiltration, splenomegaly and anaemia of chronic disease)

Question 6

How do we investigate patients with lymphoma?

Answer 6

• History: symptoms and duration
• Clinical examination: lymph nodes, splenomegaly, hepatomegaly
• Bloods: FBC, UEs, LFTs, Ca, LDH (ca and ldh = aggressive), urate as can precipitate gout
• Imaging: CT/PET scan
• Bone marrow biopsy
• Echocardiogram as drugs may affect the heart

Question 7

Describe the investigations carried out for staging of lymphoma

Answer 7

• CT of neck, chest, abdomen and pelvis
• PET/CT - diffuse large B cell lymphoma or follicular lymphoma
• Bone marrow aspirate and biopsy

Question 8

What investigations into lymphoma are to help determine fitness for treatment?

Answer 8

• Renal function
• Liver function
• Bone marrow function
• Cardiac disease
• Respiratory disease

Question 9

What system is used for the classification of lymphoma?

Answer 9

Ann-Arbor classification system

Question 10

Stage 1 lymphoma

Answer 10

Single lymph node group (above or below the diaphragm)

Question 11

Stage II lymphoma

Answer 11

More than one lymph node group, all on the same side of the diaphragm

Question 12

Stage III lymphoma

Answer 12

Lymph node groups on both sides of the diaphragm

Question 13

Stage IV lymphoma

Answer 13

Extranodal involvement e.g. liver, bone marrow, lungs

Question 14

What does the A/B mean in the classification of the staging of lymphoma?

Answer 14

•Presence of B symptoms of not

Question 15

Give an example of an indolent B cell lymphoma

Answer 15

Follicular lymphoma

Question 16

Give an example of an aggressive B cell lymphoma

Answer 16

• Diffuse large B cell lymphoma

* Burkitt’s lymphoma

Question 17

What is the antigen expressed on the B-lymphocytes?

Answer 17

CD20

Question 18

What is the treatment of B cell lymphoma?

Answer 18

Rituximab

Question 19

Describe follicular lymphoma

Answer 19

• B cell
• Resemblance to normal germinal centres
• Translocations involving BCL2 gene
• Slow growth but reduced cell death
• usually incurable and tends to present late at stage 4

Question 20

What is the treatment of follicular lymphoma

Answer 20

•If stage I or II may be cured by radiotherapy
•Incurable for majority
•Treatment in advanced stage is aimed at alleviating symptoms and preventing end organ compromise
•Only treat if symptomatic in advanced disease:
- immune-chemotherapy: anti CD20 (rituximab)
- chemotherapy (CHOP or CVP or Bendamustine)

Question 21

What is the natural history of follicular lymphoma?

Answer 21

• Very responsive to treatment but tendency to relapse

* May transform to diffuse large B cell lymphoma

Question 22

Describe large B cell lymphoma

Answer 22

• Resemblance to activated B cells (immunoblasts and centroblasts)
• Heterogeneous entity: variable phenotype
• Associated with various translocations and genetic abnormalities, complex karyotype
• High proliferation fraction, variable rate of cell death

Question 23

What is the most common subtype of NHL?

Answer 23

Diffuse Large B cell lymphoma

Question 24

What is the presentation of diffuse large cell lymphoma?

Answer 24

•Lymphadenopathy 
•Usually rapidly enlarging 
•Lymph node mass 
•Extra nodal presentation is common 
 - waldeyer's ring 
 - GI tract
 - skin 
 - bone 
 - CNS etc 
•Pyrexia of unknown origin 
•Night sweats and weight loss 
•Aggressive but curable in >50%

Question 25

What are the investigations for diffuse large B cell lymphoma

Answer 25

• CT scan
• PET/CT scan
• Bone marrow biopsy
• Blood tests
• Echocardiogram

Question 26

What is the treatment of diffuse large B cell lymphoma

Answer 26

• Requires aggressive chemotherapy with intention to cure
• Response is variable; subset are resistant to conventional treatments
• Early stage (1A): R-CHOP chemotherapy x3 with radiotherapy
• For all other stages R-CHOP x6
• Needs adequate cardiac function (doxorubicin)

Question 27

What is R-CHOP?

Answer 27

• Rituximab
• Cyclophosphamide
• Doxorubicin
• Vinvristine
• Prednisolone

Question 28

What is the most common high grade lymphoma in kids?

Answer 28

Burkitt lymphoma

Question 29

What is the translocation in Burkitts lymphoma?

Answer 29

8:14

Question 30

What are the characteristics of burkitt lymphoma?

Answer 30

• Resemblance to proliferating germinal centre cells (histologically)
• Characterised by translocations involving MYC gene
• Very high rate of proliferation (nearly all cells in the cell cycle)
• High rate of cell death (apoptosis)

Question 31

Describe the presentation of burkitt lymphoma

Answer 31

•Marked b symptoms
•Rapidly growing tumour with massive tumour bulk
•Most cases present with extra nodal disease
- jaws and facial bone
- ileocaecal region of GIT
- ovaries
- kidney
- breast
- lymph nodes and bone marrow more frequently affected in immunosuppression associated BL
- CNS involvement at presentation or relapse is common in all types (brain, meninges or both)