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lymphoma
lymphoma
see also:
neoplasia
EBV / glandular fever / infectious mononucleosis
introduction
85% of malignant lymphomas are non-Hodgkins lymphomas (NHL)
median age at diagnosis is in 6th decade with exception of Burkitt lymphoma and lymphoblastic
lymphoma which occur in younger patients
Hodgkin's lymphoma
accounts for 12% of cases of lymphoma
most patients are young adults (20-35yrs old) or in the 70's.
5 year survival with Rx:
stage I/II 90%
stage III 84%
stage IV 65%
~50% contain EBV / glandular fever / infectious mononucleosis genome, esp. in:
patients with mixed cellularity tumours
young children
elderly
risk factors
genetic:
twin with HL ⇒ 99x risk1)
parent or sibling with HL or any blood or lymphatic cancer ⇒ 7x risk
infectious mononucleosis infection ⇒ 3x risk
immunosuppression:
HIV/AIDS ⇒ 11x risk
organ transplantation
autoimmune diseases such as:
rheumatoid arthritis, systemic lupus erythematosus and sarcoidosis
childhood affluence (presumably via delay in acquiring EBV, or less childhood infections
resulting in altered immunity)
PH Non-Hodgkin's lymphoma ⇒ 4-16x risk
some alcohol consumption appears to reduce risk as does being breastfed
clinical features
painless and slowly progressive, rubbery lymphadenopathy
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80% are above the diaphragm, 60-80% involve neck, <20% axillary, <20% groin
<10% have pain at nodal sites precipitated by drinking alcohol
if massive mediastinal nodes, may develop superior vena cava syndrome
extranodal involvement
splenomegaly
hepatosplenomegaly
40% have B symptoms
T > 38°C, night sweats
weight loss >10% from baseline within 6 months
fatigue/weakness
35% have intermittent fever
some have classic Pel-Ebstein fever
high fever for 1-2 wks followed by an afebrile period of 1-2 wks
pruritus
chest pain, cough &/or SOB
paraneoplastic syndromes
rarely:
bone/back pain
haemoptysis
non-Hodgkin's lymphoma
85% of NHLs are of B-cell origin
15% are derived from T/NK cells
the remainder arise from macrophages
5 year survival is just over 60%
risk factors
EBV / glandular fever / infectious mononucleosis + malaria ⇒ endemic Burkitt's lymphoma in
children
EBV / glandular fever / infectious mononucleosis + immunosuppression
⇒ post-transplant lymphoma
13% of heart, and 33% of heart/lung recipients have been reported to develop NHL
The latest meta-analysis showed an 8-fold increased risk of NHL in transplant
recipients
⇒ HIV-related lymphoma
HIV is 80x risk with 3-5% developing NHL and HIV accounts for 6% of NHL cases in
developed countries
EBV is present in 2/3rds of AIDS-related NHL
strong association between Helicobacter pylori and lymphomas of mucosa-associated lymphoid
tissue (MALT) in the stomach
4% of NHL cases in developed countries are due to infection with H.pylori
Hepatitis C virus infection ⇒ 2-2.5x risk
Human T-Cell Lymphoma Virus 1 causes a small number of cases of NHL in developed countries
auto-immune conditions
haemolytic anaemia, systemic lupus erythematosus and Sjogren syndrome
coeliac disease is associated with an increased risk of T-cell lymphoma specifically
genetic:
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lymphoma
FH NHL ⇒ 2x risk
FH coeliac disease in a sibling ⇒ increased risk
carcinogens:
A meta-analysis of case-control studies reported a 35% increase in risk for people with
occupational exposure to pesticides, increasing to 65% for more than 10 years' exposure
Occupational exposure to benzene has been linked to a 22% increase in NHL risk in a
meta-analysis
obesity:
a 40% increased risk of diffuse large B-cell NHL for people with a BMI of 30 or higher.
sub-types of NHL
Anaplastic Large Cell Lymphoma
Follicular Lymphoma
B Cell Lymphoma
Diffuse Large Cell Lymphoma
Diffuse Mixed Lymphoma
Lymphoblastic Lymphoma
Mantle Cell Lymphoma
has the worst prognosis of the B-cell lymphomas
characterised by over-expression of cyclin D1 and the dysregulation of the cell cycle
Mycosis Fungoides
Primary CNS Lymphoma
Cutaneous T-Cell Lymphoma
clinical features
painless and slowly progressive lymphadenopathy
cytopenias
with advanced stages of low grade tumours, or early stages of high grade tumours:
extranodal involvement
esp. GI/GU tracts (including Waldeyer ring), skin, bone marrow, sinuses, thyroid,
CNS
splenomegaly
hepatosplenomegaly
testicular mass
skin lesions (mycosis fungoides)
B symptoms
T > 38°C, night sweats
weight loss >10% from baseline within 6 months
fatigue/weakness
1)
Cancer Research UK
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