Lymphoma

Question 1

What is the definition of lymphoma?

Answer 1

A neoplastic tumour of lymphoid tissue, often in lymph nodes. But they can spill out into the BM, blood and other parts of the body

Question 2

What are the different kinds of lymphoma?

Answer 2

There are around 60 types

Hodgkin’s Lymphoma (20%)

Non-Hodgkin's Lymphoma (80%)
T cell
- Adult T cell Leukaemia/Lymphoma (ATLL)
- Peripheral T-cell Lymphoma
- Enteropathy-associated T cell Lymphoma (EATL)
- Cutaneous T cell Lymphoma
- Anaplastic Large cell Lymphoma

B cell

• Burkitt’s
• Diffuse Large B cell (DLBC)
• Mantle cell
• Follicular
• Mucosal associated Lymphoid Tissue (MALT)

Question 3

What is the epidemiology surrounding Hodgkin’s lymphoma?

Answer 3

There is a bimodal peak at 20-29 and >60 years old, with males more at risk than females. It associated to EBV infection and usually spreads between lymph nodes

Question 4

What are the different types of Hodgkin’s lymphoma?

Answer 4

Classical:

• Nodular sclerosing
• Mixed cellularity
• Lymphocyte rich

Lymphocyte predominant

Question 5

What is the clinical presentation of Hodgkin’s lymphoma?

Answer 5

Asymmetrical painless lymphadenopathy +/- mass symptoms
B symptoms:
- Fever (often cyclical)
- Night sweats
- Weight loss of >10% in 6 months
Pain in nodes after alcohol

Question 6

What investigations are ordered for Hodgkin’s lymphoma?

Answer 6

• CT PET for staging
• LN or BM biopsy (Reed-Sternberg cells - bi nucleate)
• Biopsy helps with subtypes

Question 7

What is the staging of Hodgkin’s lymphoma?

Answer 7

Stage 1: A single node
Stage 2: Multiple nodes, one side of diaphragm
Stage 3: Multiple nodes, either side of diaphragm
Stage 4: outside of nodes

A: no B symptoms
B: B symptoms present

Question 8

What is the treatment of Hodgkin’s lymphoma?

Answer 8

1) Combination chemotherapy
- ABVD: preserves fertility
- 2-4 cycles or 6-8 cycles depending on stage

2) Radiotherapy
- Side effects of cancers in targeted areas

3) Intensive chemotherapy
- If SCT is available, for relapsed patients

Question 9

Describe the stem cell transplant options available for lymphoma patients

Answer 9

Autologous SCT:

• Own stem cells frozen and reintroduced
• No GVHT, no infection, no malignancy

Allogenic SCT:

• HLA matched donor stem cells introduced
• GVHD risk, infection risk, secondary malignancies

Question 10

How can non-Hodgkin’s lymphomas be classified?

Answer 10

According to cell type (B or T)
According to maturity of cell type
According to histology

Question 11

What are the clinical features of non-Hodgkin’s lymphoma?

Answer 11

These can vary dramatically, but there are similarities:

• Painless lymphadenopathy
• No pain after alcohol
• Staging as per Hodgkin’s

Question 12

Describe T cell lymphomas

Answer 12

These are rarer than B cell lymphomas

Peripheral T cell Lymphoma

• Middle aged and elderly patients
• Very aggressive
• Large T cells

Adult T cell Leukaemia/Lymphoma

• Caribbean and Japanese patients
• HTLV-1 infection associated

Enteropathy associated T cell Lymphoma
- Longstanding coeliac disease

Cutaneous T Cell Lymphoma
- Mycosis fungoides

Anaplastic Large cell Lymphoma

• Children and young adults
• Aggressive
• Large epithelioid lymphocytes
• t(2;5), Alk-1 protein expression

Question 13

Describe the different types of Burkitt’s lymphoma

Answer 13

This is a B cell lymphoma; all are aggressive and fast growing with a t(8;14) but responsive to treatment; histology shows a starry night appearance. Treatment is with chemotherapy (rituximab) or SCT.

Endemic

• Most common in equatorial Africa
• EBV associated
• Jaw involvement and abdominal masses

Sporadic

• Found outside Africa
• EBV associated
• Jaw involvement rare

Immunodeficiency

• Non EBV associated
• HIV associated/ post transplant

Question 14

Describe other (not Burkitt’s) B cell lymphomas (epidemiology, presentation, features), mention what might show on histology, and what treatment is offered

Answer 14

Diffuse Large B cell (DLBC)

• Middle ages and elderly
• Aggressive
• Richter’s transformations on histology (sheets of large lymphoid cells)
• Other types are secondary to DLBC
• Treatment with chemotherapy (rituximab)

Mantle cell Lymphoma

• Middle aged, more in males
• Aggressive
• Presents when disseminated
• Bad prognosis (3-5 years)
• t(11;14)
• Histology shows angular nuclei
• Treatment with chemotherapy (rituximab)

Follicular

• Elderly
• Mostly curable
• 12-15 years survival
• t(14;18)
• Follicular pattern or nodular appearance on histology
• Watch and wait, offer rituximab

Mucosal Associated Lymphoid Tissue (MALT)

• Marginal zone lymphoma
• Middle aged
• Antigen stimulation (H. pylori, Sjogren’s syndrome)
• Treatment by removing antigen stimulus (antibiotics, chemotherapy)