Lymphoma Flashcards
What is lymphoma
Neoplastic disorder of lymphocytes (T or B cells) in lymphoid tissue. This can be in the lymph nodes (nodal) or extra nodal (could be things like MALT or the tonsils)
Incidence - Sharply increases with age and slightly more common in men.
What is the basic classification of lymphoma
Non-Hodgkin’s - further divided into B cells or T cells then indolent or aggressive.
Hodgkin’s - Classical or nodular lymphocyte predominant
Explain the presentation of lyphoma
- Lymphadenopathy (painless and rubbery)
- Splenomegaly
- Extranodal disease (breastm brain, lung)
- B symptoms (Night sweats, weight loss and unexplained fever),
- Anaemia (Bone marrow infiltration, splenomegaly and ACD)
What are the B symptoms?
Night sweats, weight loss and unexplained fever
What is the investigations for a patient with lymphoma?
History - symptoms and duration, presence of B symptoms.
Clinical exam - Lymph nodes, spleno/hepato-megal.
Blood tests - FBC, U&Es, LFTs, Ca, lactate dehydrogenase and urate.
Imaging - CT of head, neck, abdo and pelvis/PET
Bone marrow biopsy - Aspirate & Trephine
Extra tests - ECG to assess heart function before being put on certain drugs
What is the function of all the blood tests when investigating a lymphoma?
Assessing fitness of patient for specific treatments
Describe the staging of lymphoma
Stage 1 - Single lymph node group,
Stage 2 - More than one lymph node group on the SAME side of the diaphragm.
Stage 3 - Lymph node involvement on BOTH sides of the diaphragm.
Stage 4 - Extranodal involvement (bone marrow or liver etc)
A or B is added to signify presence or absence of B symptoms. This is the same for hodgkin’s and non-hodgkins
What are some factors affecting treatment decisions for lymphoma?
Type of lymphoma, if patient is symptomatic, stage of lymphoma, age and performance status, comorbidities and support
Name examples indolent and aggressive B cell non- hodgkins lymphoma
Indolent - Follicular lymphoma
Aggressive - Diffuse large B cell lymphoma and Burkitt’s lymphoma
Describe features of follicular lymphoma
- Resembles a lymph node germinal centre
- Caused by translocations involving the BCL2 gene.
- Slow growing and often presents with stage 4 disease which is usually incurable
- Commonest low grade lymphoma in adults. Often older patients affected
What are the investigations for follicular lymphoma?
- CT scan,
- PET/CT scan if CT suggests localised disease.
- Bone marrow biopsy if planning to treat,
- Blood tests,
- ECG if planning for R-CHOP
What are the principles of treatment for follicular lymphoma
- Majority is incurable (early stage may be cured by radiotherapy.
- Treatment in advanced is normally alleviating symptoms and preventing end organ compromise. If asymptomatic then wait and watch.
- Treatment if symptomatic/bulky disease/end-organ compromise: Immuno-chemo therapy with rituximab and CVP/CHOP/Bendamustine.
Followed by rituximab every 2 months for 2 years
What antigen is on the surface of B-lymphocytes and how is this targeted in treatment?
CD20 antigen. It is targeted with the monoclonal antibody - Rituximab
Describe features of diffuse large B-cell lymphoma
It resembles activated B cells and it is heterogenous and is associated with various translocations and genetic abnormalities. May express CD10 or BCL2.
Mainly occurs in adults but can occur in children. >50% can be cured but depends on stage, prognostic score and co-morbidities
What is the presentation of diffuse large B cell lymphoma
Wide variation in presentation but can present with lymphadenopathy and extra nodal presentation (Waldeyer’s ring, GI tract, skin, bone, and CNS), also presents with pyrexia of unknown origin, night sweats and weight loss.
Very aggressive but curable.
What are the investigations and treatment for DLBCL
Investigations - CT scan, PET/CT scan, bone marrow biopsy, blood tests and ECG.
Treatment - Aggressive chemo. If early stage (1A) then 3 rounds of R-CHOP and radiotherapy. If a larger stage then 6 rounds of R-CHOP. However need adequate cardiac function for doxorubicin. Careful in eldery/unfit patients
What drugs are used in R-CHOP chemotherapy?
R- Rituximab
C - Cyclophosphamide
H - Doxorubicin
O - Vincristine,
P - Prednisolone
Describe features of Burkitt Lymphoma
- Commonest high grade lymphoma in children.
- Resembles a proliferating germinal centre.
- Characterised by translocation in the MYH gene.
- High rate of cell proliferation but also high rate of apoptosis (high risk of tumour lysis syndrome)
- Requires intensive chemotherapy
How can Burkitt lymphoma present?
- Usually short history, presence of B symptoms and rapidly growing tumour.
- Most have extranodal presentation with tumours in Jaws/facial bones, ileocecal region in GI, ovaries, kidneys, breast, lymph node and bone involvement in immunosuppression- associated BL, and CNS involvement
What are the different types of classical Hodgkin’s lymphoma?
- Nodular sclerosing,
- Mixed cellularity,
- Lymphocyte rich,
- Lymphocyte depleted
Describe features of classical hodgkin’s lymphoma
- Neoplastic cells (Reed Sternberg cells) resemble atypical activated B-cells as seen in some viral infections.
- Cells express CD30 and have some loss of B cell antigens.
- 40% cases associated with EBV infection.
What is the incidence of Hodgkin’s lymphoma?
More common in males and has a bimodal age distribution with first peak in young adults and second peak in late 70s.
What is the presentation of Hodgkin’s lymphoma?
- Painless rubbery lymphadenopathy,
- Cough and SOB.
- B symptoms,
- Itch which may precede diagnosis for months,
- Alcohol related pain but this is very rare
What are the investigations for Hodgkin’s lymphoma?
- History,
- Clinical exam (Lymph nodes, liver and spleen)
- Blood tests (HBC, U&Es, LFTs, ESR and LDH)
- Imaging (CT and PET/CT. Pet is useful for detecting extranodal disease),
- No longer require bone marrow biopsy,
- Pre-treatment tests such as ECG and pulmonary function tests
What is the treatment for Hodgkin’s lymphoma?
- Early stage is chemo (ABVD) followed by radiotherapy.
- Advanced stage is chemo (ABVD)
What is ABVD chemotherapy?
A - Adriamycin (Doxorubicin),
B - bleomycin,
V - vinblastine,
D - dacarbazine.
Can remove bleomycin after cycle 2 if PET scan is neg
What is the prognosis for Hodgkin’s lymphoma
High cure rates but dependant on stage of disease and fitness of patient. If caught early then >90% cure rate. Advance staged is 75-85%. However cure rate in elderly with advanced is poor