Non-Hodgkin's Lymphoma Flashcards
What is NHL?
Malignant tumours of lymphoid tissue that do not contain Reed-Sternberg cells
What percentage are B cell in origin and what percentage T cell?
70% are B cell in origin
30% T cell
Is it the most common haematopoietic malignancy?
Yes
What does the incidence increase with?
Age
What is the median age of presentation?
55-75
Do all the conditions involve lymph nodes?
No - extranodal generating lymphoma including mucosa-associated lymphoid tissue e.g gastric MALT Spleen BM Thymus Oropharynx
Is there a slight male or female predominance?
Male
What risk factors are there?
Elderly, Caucasians
History of viral infection specifically EBV
FH
Certain chemical agents - pesticides, solvents
History of chemotherapy or radiotherapy
Immunodeficiency- transplant, HIV, DM
Autoimmune disorders- SLE, Sjogrens, coeliac, RA..
How can NHL be classified?
Low grade - B cell or T cell
High grade - B cell or T cell
Is HL or NHL more common?
NHL
What symptoms and signs are associated?
Superficial, painless, rubbery lymphadenopathy
B symptoms - weight loss, fever, night sweats
Constitutional symptoms - lethargy, pruritis
Extranodal involvement:
GI - abdominal pain, dyspepsia, constipation or diarrhoea
Oropharynx (Waldeyer’s ring) - dysphagia, obstructed breathing
BM - fatigue, bleeding and bruising, recurrent infections, bone pain
Lungs - SOB, cough
Skin - red patches or scaly lumps under skin
CNS - headaches, seizures, weakness/numbness
Hepatosplenomegaly
Do B symptoms typically occur earlier in HL or NHL?
Earlier in HL
B symptoms in NHL suggest disseminated disease
Is extra nodal disease more common in NHL or HL?
Much more common in NHL
What lymph nodes are typically affected?
Cervical
Axillary
Inguinal
What is the diagnostic investigation of choice?
Excisional node biopsy NOT FNA
What imaging is needed?
CTCAP to assess staging
What tests should be done?
FBC and blood film
U&E
Uric acid - some aggressive NHLs can cause high levels
LFTs - if liver mets expected
ESR - prognostic indicator
LDH - a marker for cell turnover, useful as a prognostic indicator
Bone marrow aspiration and trephine are routine
HIV test - this is a RF for NHL
LP if CNS involvement
What staging system is most commonly used?
Ann Arbor
Describe low grade lymphomas
Indolent, often incurable and widely disseminated
Follow a replacing and remitting course
Median survival = 10 years
Accounts for 45% of cases
How can B and T tumours be distinguished?
Immunophenotyping- dome on blood, marrow or nodal material
What types of low grade lymphoma are there?
Follicular lymphoma
Marginal zone / MALT
Lymphocytic lymphoma - closely related to CLL
Hairy cell leukaemia
Describe high grade lymphoma
More aggressive
More likely to be cured
Often rapidly enlarging lymphadenopathy with systemic symptoms
80% will initially respond to treatment
What types of aggressive NHLs are there?
Diffuse large B cell = most common
Burkitt’s lymphoma
Lymphoblastic lymphoma
Mantle cell
Describe Burkitt’s lymphoma
Commonly seen in West Africa Childhood disease Highly aggressive Associated with jaw lymphadenopathy Associated with EBV
What is the link between Burkitt’s lymphoma and malaria?
Malaria thought to reduce resistance to EBV, allowing the virus to take hold
How is low grade type treated?
Depends on sub type present
If symptomless - likely no treatment given and monitored
RT may be curative if localised disease
Chlorambucil (chemotherapy) can be used if diffuse
Remisssion may be maintained with alpha interferon or rituximab
How is high grade lymphoma treated?
Chemotherapy regime: R-CHOP Rituximab Cyclophosphamide Hydroxydaunorubicin Vincristine (Oncovin) Prednisolone
What can be given to help neutropenia?
GCSF
What suggests poor prognosis?
Over 60
Bulky disease e.g abdominal mass > 10cm
Raised LDH
Disseminated disease
Rituximab kills what cells?
CD20+
Some lymphomas can produce IgM leading to what?
Hyperviscosity syndrome - IgM is the biggest gamma globulin
Autoimmune cytopenias e.g AIHA
Some lymphomas produce very few gammaglobulins. What does this lead to?
Infections