Lymphomas

Question 1

What is Hodgkin’s lymphoma?

Answer 1

Malignant proliferations of lymphocytes which accumulate in the lymph nodes causing lymphadenopathy and can also be found in peripheral blood and organs. Hodgkin’s lymphoma has characteristic cells with mirror image nuclei called Reed-Sternberg cells. Most often T cell line.

Question 2

What are the risk factors for lymphomas?

Answer 2

Two ages peaks – 14-24 and elderly 
2:1 Male to female ratio 
Affected sibling 
EBV 
SLE 
Post transplantation

Question 3

What are the clinical features of lymphoma?

Answer 3

Enlarged non tender rubbery superficial lymph nodes (most commonly cervical but also axillary or inguinal)
Fever, weight loss, night sweats, pruritis and lethargy
Alcohol induced lymph node pain
Mediastinal lymph node involvement can cause systemic upset e.g. bronchial or SVC obstruction and pleural effusions, so presentation with cough and shortness of breath
Spleno or hepatomegaly
Cachexia

Question 4

How should suspected lymphoma be investigated?

Answer 4

FBC
Blood film
ESR
Bone marrow biopsy and aspiration
LFTs Us and Es and LDH (including urate) and calcium studies
Lymph node excision biopsy or Image guided needle aspiration
Immunological and cytogenetic analysis

CXR
CT/PET of thorax, abdo and pelvis

Question 5

Describe the Ann Arbor staging of lymphoma?

Answer 5

Ann Arbor staging

1. Confined to single lymph node region
2. Involvement of two or more nodal areas on the same side of the diaphragm
3. Involvement of nodes on both sides of the diaphragm
4. Spread beyond the lymph nodes e.g. liver or bone marrow

Then either A if only asymptomatic or B for B symptoms: weight loss (>10% over 6months), unexplained fever >38, night sweats. Localised extra nodal extension does not advance the stage but add subscript E on the end e.g. 3BE

Question 6

How is Hodgkin’s lymphoma managed?

Answer 6

Radiotherapy and short chemo courses for stages 1A and 2A (<3 areas)
Longer chemo courses for 2A (>3 areas) through to 4B
Achieves 80% cure rate
In advanced disease or relapse high dose chemo followed by autologous stem cell transplant

Question 7

What is Non-Hodgkin’s lymphoma?

Answer 7

This includes all lymphomas without reed Sternberg cells. Majority are derived from B cell lines with Diffuse B-cell lymphoma being the most common. Not all will centre around lymph nodes with some associating with mucosa associated lymphoid tissue e.g. Gastric MALT.

Question 8

What are the risk factors for Non-hodgkin’s lymphoma?

Answer 8

Immunodeficiency drugs 
HIV (usually high grade from EBV transformed cells) 
HTLV-1 (oncogenic virus) 
H-pylori 
Toxins 
Congenital

Question 9

Describe the clinical features of Non-hodgkin’s lymphoma?

Answer 9

Superficial Lymphadenopathy  
Extranodal disease (gut most common) 
1.	Gastric MALT due to H-pylori
     o	May regress with eradication of H-pylori, typically in the antrum, multifocal and metastasises late. 
     o	Non-MALT gastric lymphoma – usually Diffuse B cell lymphoma, are diffuse and don’t respond well to H-Pylori eradication 
2.	Small bowel lymphomas 
3.	Skin 
4.	Oropharynx 

Systemic features such as night sweats, weight loss and fever
Pancytopenia from marrow involvement
Splenomegaly especially low-grade disease

Question 10

How should Non-Hodgkin’s lymphoma be investigated?

Answer 10

FBC
Blood film
Bone marrow biopsy and aspiration
Us + Es, LFTs and LDH
Lymph node biopsy either from excision or image guided needle
Ann Arbor staging so CT/PET of chest abdomen and pelvis
Lumbar puncture if CNS signs

Question 11

What are low grade vs high grade non-hodgkin’s lymphomas?

Answer 11

Low-grade lymphomas are disseminated and incurable and most common in middle/old aged men.

High-grade lymphomas are more aggressive

Question 12

How are low grade non-Hodgkin’s lymphomas treated?

Answer 12

If advanced no management is needed at all especially if no symptoms. Radiotherapy can be used in localised disease. Chemotherapy can be used specifically biologicals such as Rituximab to maintain remission.

Question 13

How are high grade Non-Hodgkin’s lymphomas treated?

Answer 13

High-grade lymphomas are more aggressive but curable e.g. large B-cell lymphoma and diffuse large B cell lymphoma, also Burkett’s Lymphoma (childhood with jaw lymphadenopathy). They present at all ages. Chemotherapy used to treat also give granulocyte colony stimulating factors to help with neutropenia.

Question 14

What are the poor prognostic factors for non-hodgkin’s lymphoma?

Answer 14

Worse if: age >60, systemic symptoms, bulky disease, raised LDH.

Question 15

What is Burkett’s Lymphoma?

Answer 15

Burkett’s Lymphoma – occur in children around the equator causing lymphadenopathy on the jaw. Treat with cyclophosphamide.