Lymphoma Flashcards
What is lymphoma?
A malignant growth of WBC’s predominantly in the lymph nodes.
A lymphoproliferative disorder – cancerous cells proliferate within lymph nodes inside the lymphatic system + cause them to enlarge abnormally
Difference between lymphomas and leukaemias?
Lymphomas are SOLID, leukaemias are CIRCULATING.
Dx confirmed by tissue sampling.
Although predominantly in the lymph nodes, lymphoma is systemic.
What other organs might it effect?
- Blood.
- Liver.
- Spleen.
- Bone marrow.
Give 4 risk factors for lymphoma.
- Primary immunodeficiency.
- Secondary immunodeficiency e.g. HIV.
- Infection e.g. EBV, HTLV-1, H.pylori.
- Autoimmune disorders e.g. RA, SLE.
Describe the pathophysiology of lymphoma.
There is impaired immunosurveillance and infected B cells escape regulation and proliferate. (This is just a theory).
Give 4 symptoms of lymphoma.
- Enlarged lymph nodes in arm/neck.
- Symptoms of compression syndromes.
- General systemic ‘B’ symptoms e.g. weight loss, night sweats, malaise.
- Liver and spleen enlargement.
What investigations might you do in someone who you suspect has lymphoma?
- Lymph node excision / biopsy
- Blood films
- Imaging - CT, MRI and PET scans
- Immunophenotyping.
- Cytogenetics.
What are the two sub-types of lymphoma?
- Hodgkins lymphoma.
- Non-hodgkins lymphoma.
What are the 2 types of Hodgkin’s lymphoma?
- Classical Hodgkin’s lymphoma (cHL)
* The hallmark of which is the REED-STERNBERG CELL with mirror image nuclei
* Accounts for 90-95% of cases - Nodular lymphocyte predominant Hodgkin’s Lymphoma (NLPHL)
* Characterised by the Reed-Sternberg VARIANT, the ‘POPCORN CELL’
Give 3 risk factors for Hodgkin’s lymphoma.
- EBV infection (glandular fever)
- HIV
- Autoimmune conditions e.g. SLE, RA, sarcoidosis
- Fx – affected sibling
- Post-transplantation
- Obesity
- Male sex predominance
Describe the epidemiology of Hodgkin’s lymphoma.
- Bimodal age = 2 peaks of incidence (teenagers and elderly – 13-19 and >65)
What are the symptoms of Hodgkin’s lymphoma?
- Painless lymphadenopathy.
- Presence of ‘B’ symptoms e.g. night sweats, weight loss, fever.
- Hepatosplenomegaly.
What features of a Hx would suggest Hodgkin’s lymphoma?
- Painless supraclavicular or cervical lymphadenopathy (may spontaneously increase/decrease in size)
- Risk factors
- B symptoms (25%)
- Pruritus (10%)
- Alcohol induced pain - LOOK out for in questions - causes pain in the lymph nodes!! - SPECIFIC
- Symptoms of mediastinal adenopathy (dry cough, dyspnoea, chest pain, SVC syndrome)
Describe the lymphadenopathy seen in Hodgkin’s lymphoma.
Enlarged, painless, rubbery superficial lymphadenopathy.
Most commonly presents with cervical or supraclavicular painless lymphadenopathy
“but I do get pain in my neck when I’ve had a drink doctor…”
What investigations would you carry out in Hodgkin’s lymphoma?
What results would you expect?
- Lymph node excision / biopsy – diagnostic!!!
- Reed-Sternberg cells (popcorn cells)
o Abnormally large B cells that have multiple nuclei that have
nucleoli inside them.
o This can give them the appearance of the face of an owl
with large eyes.
- Reed-Sternberg cells (popcorn cells)
- Bloods
* High ESR or Low Hb - indicate worse prognosis
* High serum lactate dehydrogenase
* Neutrophilia - Imaging – CT, MRI and PET scans
* Of the chest, abdomen + pelvis to see at which stage - Immunophenotyping
- Cytogenetics
What is needed for diagnosis of Hodgkins lymphoma?
Presence of Reed-sternberg cells.
Describe the staging of Hodgkins lymphoma.
Used for both HL and NHL.
The system puts importance on whether the affected nodes are above or below the diaphragm.
Each stage is either A or B:
* A: no systemic symptoms other than pruritus (severe itching of skin)
* B: presence of B symptoms such as fever, weight loss and night sweats
Stage 1: Confined to 1 region of lymph nodes.
Stage 2: In > 1 region but on the same side of the diaphragm (either above or below).
Stage 3: Affects lymph nodes both above and below the diaphragm.
Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.
How would you manage Hodgkin’s lymphoma?
Combination chemotherapy: ABVD
A – Adriamycin
B – Bleomycin
V – Vinblastine
D – Dacarbazine
What is the treatment for stage 1 - 2A Hodgkins lymphoma?
Short course of ABVD followed by radiotherapy
What is the treatment for stage 2B - 4 Hodgkins lymphoma?
Longer course of ABVD (combination chemotherapy)
What are the possible complications of treatment for Hodgkin’s lymphoma?
- Secondary malignancies.
- IHD.
- Infertility.
- Nausea.
- Alopecia.
What complications can chemotherapy for HL cause?
- Chemotherapy -> creates a risk of leukaemia and infertility.
What complications can radiotherapy for HL cause?
- Radiotherapy -> creates a risk of cancer, damage to tissues and hypothyroidism.
What are complications of HL?
- Myelosuppression and neutropenic sepsis.
- Tumour lysis syndrome.
- Impaired immunity.
What are Non-Hodgkin’s lymphomas?
Includes all lymphomas without Reed-Sternberg cells
- 80% = B cell origin
- 20 % = T cell origin
- Not all centre on nodes
What histological feature differentiates HL from NHL?
Presence of Reed-Sternberg cells (large multinucleated mirror-image nuclei) in HL
(They look like little owls)
What AIDS defining illness is associated with NHL?
Burkitt’s lymphoma - jaw lymphadenopathy
List some subtypes of NHL.
1) Diffuse large B-cell lymphoma (30%) - aggressive/high grade
2) Follicular lymphoma (20%) - indolent/low grade
3) Burkitt’s (HG) (1%) - EBV - characteristic jaw lymphadenopathy (child)
4) Primary CNS lymphoma (1%) - EBV with AIDS
Also:
5) MALT (LG) (gastric mucosa associated lymphoid tissue) - H.pylori
6) Marginal zone - massive splenomegaly
7) T cell
What conditions is NHL associated with?
Autoimmune disorders - Sjogrens, RA, coeliac, SLE.
Immunodeficiency.
Describe low grade non-hodgkins lymphoma.
Slow growing, advanced at presentation, often incurable.
Median survival is 10 years.
Describe high grade non-hodgkins lymphoma.
Aggressive. Nodal presentation, patient unwell.
Often curable.
How does NHL present? Give some key signs + symptoms.
1) Nodal signs - lymphadenopathy (can be rapid)
2) Extranodal signs:
- Bone marrow - pancytopenia (fatigue, dyspnoea)
- Splenomegaly (massive in marginal zone) + hepatomegaly
- Dry cough - mediastinal mass/pneumonia
- Skin (T cell)
- Gut: diarrhoea, vomiting, abdominal pain (MALT)
- Bone - bone pain
- CNS
3) B symptoms (less common than Hodgkin’s)
- Night sweats
- Fever
- Weight loss > 10%
What investigations would you do in NHL?
What would you expect to see?
- FBC
- Raised ESR/LDH ( lactose dehydrogenase) - reflects worse prognosis since its a sign of increased cell turnover and thus cell proliferation
- Normocytic anaemia, thrombocytopenia or pancytopenia - Lymph node excision or bone marrow biopsy
- Will NOT see mirror-image nuclei REED-STERNBERG CELLS or Reed-Sternberg variant cells - POPCORN CELLS - Marrow and node biopsy
- For classification - CT/MRI
- Of chest, abdomen and pelvis for staging (Ann Arbor) - Immunophenotyping
- Cytogenetics
Describe the staging of lymphomas (same is used for NHL and HL)
Ann Arbor system (get a number and a letter):
1 - Single lymph node group
2- Multiple on same side diaphragm
3 - Multiple on opposite side diaphragm
4 - Multiple extranodal sites or lymph nodes and extranodal disease
E - extranodal extension
B - weight loss > 10%, fever, drenching night sweats
A - no B symptoms
B symps = worse prognosis
What combination chemotherapy regime is commonly used in patients with non hodgkin’s lymphoma?
RCHOP.
How would you manage NHL?
R-CHOP-21 regimen:
* R - RITUXIMAB (monoclonal antibody - minimal side effects)
* C - CYCLOPHOSPHAMIDE
* H - HYDROXY-DAUNORUBICIN
* O - VINCRISTINE (Oncovin brand name)
* P - PREDNISOLONE for 21 days
What is the treatment for low grade NHL?
If symptomless - do nothing.
Radiotherapy, combination chemotherapy and mAb may be used if symptomatic.
Describe the treatment for high grade NHL.
- Early: short course chemotherapy and radiotherapy.
- Advanced: combination chemotherapy and mAb.
