Non Hodgkin's lymphoma Flashcards
What is a lymphoma?
A type of cancer that develops from lymphocytes, lymphoma can arise in the lymph nodes (nodal) or in other sites (extra nodal)
What are the 2 broad subtypes of lymphoma?
- Hodgkins (15%)
* Non-Hodgkin’s
What are the categories of Hodgkin’s lymphoma?
- Classical (>90%)
* nodular lymphocyte predominant
What are the categories of non-hodgkin lymphoma?
•B cell
•T cell
These categories are both further subdivided into indolent and aggerssive
How do patients with lymphoma present?
•Lymphadenopathy - either painless or rubbery
•Splenomegaly
•Extranodal disease e.g. breast, brain or lung
•B symptoms:
- night sweats
- weight loss
- unexplained fever
•Anaemia ( due to bone marrow infiltration, splenomegaly and anaemia of chronic disease)
How do we investigate patients with lymphoma?
- History: symptoms and duration
- Clinical examination: lymph nodes, splenomegaly, hepatomegaly
- Bloods: FBC, UEs, LFTs, Ca, LDH (ca and ldh = aggressive), urate as can precipitate gout
- Imaging: CT/PET scan
- Bone marrow biopsy
- Echocardiogram as drugs may affect the heart
Describe the investigations carried out for staging of lymphoma
- CT of neck, chest, abdomen and pelvis
- PET/CT - diffuse large B cell lymphoma or follicular lymphoma
- Bone marrow aspirate and biopsy
What investigations into lymphoma are to help determine fitness for treatment?
- Renal function
- Liver function
- Bone marrow function
- Cardiac disease
- Respiratory disease
What system is used for the classification of lymphoma?
Ann-Arbor classification system
Stage 1 lymphoma
Single lymph node group (above or below the diaphragm)
Stage II lymphoma
More than one lymph node group, all on the same side of the diaphragm
Stage III lymphoma
Lymph node groups on both sides of the diaphragm
Stage IV lymphoma
Extranodal involvement e.g. liver, bone marrow, lungs
What does the A/B mean in the classification of the staging of lymphoma?
•Presence of B symptoms of not
Give an example of an indolent B cell lymphoma
Follicular lymphoma
Give an example of an aggressive B cell lymphoma
- Diffuse large B cell lymphoma
* Burkitt’s lymphoma
What is the antigen expressed on the B-lymphocytes?
CD20
What is the treatment of B cell lymphoma?
Rituximab
Describe follicular lymphoma
- B cell
- Resemblance to normal germinal centres
- Translocations involving BCL2 gene
- Slow growth but reduced cell death
- usually incurable and tends to present late at stage 4
What is the treatment of follicular lymphoma
•If stage I or II may be cured by radiotherapy
•Incurable for majority
•Treatment in advanced stage is aimed at alleviating symptoms and preventing end organ compromise
•Only treat if symptomatic in advanced disease:
- immune-chemotherapy: anti CD20 (rituximab)
- chemotherapy (CHOP or CVP or Bendamustine)
What is the natural history of follicular lymphoma?
- Very responsive to treatment but tendency to relapse
* May transform to diffuse large B cell lymphoma
Describe large B cell lymphoma
- Resemblance to activated B cells (immunoblasts and centroblasts)
- Heterogeneous entity: variable phenotype
- Associated with various translocations and genetic abnormalities, complex karyotype
- High proliferation fraction, variable rate of cell death
What is the most common subtype of NHL?
Diffuse Large B cell lymphoma
What is the presentation of diffuse large cell lymphoma?
•Lymphadenopathy •Usually rapidly enlarging •Lymph node mass •Extra nodal presentation is common - waldeyer's ring - GI tract - skin - bone - CNS etc •Pyrexia of unknown origin •Night sweats and weight loss •Aggressive but curable in >50%
What are the investigations for diffuse large B cell lymphoma
- CT scan
- PET/CT scan
- Bone marrow biopsy
- Blood tests
- Echocardiogram
What is the treatment of diffuse large B cell lymphoma
- Requires aggressive chemotherapy with intention to cure
- Response is variable; subset are resistant to conventional treatments
- Early stage (1A): R-CHOP chemotherapy x3 with radiotherapy
- For all other stages R-CHOP x6
- Needs adequate cardiac function (doxorubicin)
What is R-CHOP?
- Rituximab
- Cyclophosphamide
- Doxorubicin
- Vinvristine
- Prednisolone
What is the most common high grade lymphoma in kids?
Burkitt lymphoma
What is the translocation in Burkitts lymphoma?
8:14
What are the characteristics of burkitt lymphoma?
- Resemblance to proliferating germinal centre cells (histologically)
- Characterised by translocations involving MYC gene
- Very high rate of proliferation (nearly all cells in the cell cycle)
- High rate of cell death (apoptosis)
Describe the presentation of burkitt lymphoma
•Marked b symptoms
•Rapidly growing tumour with massive tumour bulk
•Most cases present with extra nodal disease
- jaws and facial bone
- ileocaecal region of GIT
- ovaries
- kidney
- breast
- lymph nodes and bone marrow more frequently affected in immunosuppression associated BL
- CNS involvement at presentation or relapse is common in all types (brain, meninges or both)
