Haematology - Lymphoma Flashcards
What is a lymphoma?
A neoplastic tumour of lymphoid tissue
- Often LNs (+BM +/- spill out to blood)
- Sometimes other lymphoid tissue (spleen, MALT)
What is the prevalence and epidemiology of Hodgkin’s lymphoma?
20%
- M>F
- 20-29yrs + >60yrs
- EBV-associated
What is the spread of Hogkin’s lymphoma?
- Aggressive
- Spreads continuously to adjacent lymph nodes, often involves single LN group
What is the prognosis of Hodgkin’s lymphoma?
Good - mostly curable
What is the clinical presentation of Hodgkin’s lymphoma?
- Asymmetrical painless lymphadenopathy +/- obstructive/mass effect Sx
- PAIN in AFFECTED NOTES AFTER ALCOHOL
- Nodes tend to be mediastinal/cervical
What are the B Symptoms?
- Fever >38C
- Drenching sweats at night
- Weight loss >10% in 6mths (unintentional)
What are some investigations and findings for Hodgkin’s lymphoma?
- Dx: Tissue = LN/BM biopsy (cells stain with CD15 + CD30)
- CT/PET
- REED-STERNBERG CELLS (bi-nucleate/multinucleate cell on background of lymphocytes + reactive cells
What are seom subtypes of Hodgkin’s lymphoma and which is most common?
- Nodular sclerosing (most common)
- Mixed cellularity
- Lymphocyte rich
- Lymphocyte depleted
- Nodular lymphocyte predominant
How is Hodgkin’s lymphoma staged?
Ann-Arbor
What is the Ann-Arbor Staging?
- Stage 1: 1 LN region (inc. spleen)
- Stage 2: 2+ LNs on SAME SIDE OF DIAPHRAGM
- Stage 3: 2+ LNs on OPPOSITE SIDES OF DIAPHRAGM
- Stage 4: Extranodal sites (Liver, BM)
- A: No constitutional Sx
- B: Constitutional Sx (B symptoms)
What is the treatment for Hodgkin’s lymphoma?
- Combination chemotherapy (ABVD - Adraimycin, Bleomycin, Vinblastine + Decarbazine)
- Radiotherapy (V. high risk of breast cancer in women - often used alongside chemo)
- Relapsed pts: second line chemotherapy (Brentuximab/pembrolizumab/nivolumab) - may need auto/allo-SCT
What is the process of a stem cell transplant?
- Stem cells harvested from: peripheral blood, M or umbilical cord blood
- Used in leukaemia, lymphoma, MM, aplastic anaemia, MDS, sickle cell anaemia + β thalassaemia
- Works best if pt in remission as consolidation Tx to reduce relapse risk
What is an autologous stem cell transplant?
- PTs own SCs are harvested + frozen
- Enables high-dose chemo +/- radiotherapy to eradicate malignant cells
- Frozen SCs then reintroduced into pt
- No graft vs leukaemia effect
- NO GVHD (graft versus host disease)
- LOWER RISK OF INFECTION
Most commonly used in:
- MM
- Lymphoma (particularly relapse)
What is Graft Vs leukaemia effect?
Where the graft is contaminated with malignant cells
- Higher relapse rate
What is an allogenic stem cell transplant?
- HLA-matched donor SCs are harvested
- Pts own BM is completely eradicated (high-dose chemo +/- radiotherapy)
- Donor SCs introduced + colonise empty BM
- GVHD risk
- Risk of opportunistic infections
- Risk of infertility
- Risk of secondary malignancy
Most commonly used in:
- Leukaemia (Graft vs leukaemia effect)
- Myelodysplastic syndrome
What is Non-Hodgkin’s Lymphoma?
All lymphomas other than Hodgkin’s
How are Hodgkin’s lymphomas classed?
- Mature or Immature
- Lineage: T/B Cell
High grade histology:
- Very aggressive = Burkitt’s
- Aggressive = Diffuce Large B cell + Mantle Cell
Low grade histollogy:
- Indolent: Follicular, Marginal zone, Small lymphocytic
What are some similarities in the presentation of different Non-Hodgkin lymphomas?
- Painless lymphadenopathy (often multiple sites)
- Constitutional Sx
- NO PAIN AFTER ALCOHOL
What staging system is used for Non-Hodgkin’s lymphoma?
Ann- Arbor Staging
What are the 3 types of Burkitt’s lymphoma?
- Endemic
- Sporadic
- Immunodeficiency
What are some general features of Burkitt’s lymphoma?
- Very aggressive
- Fast growing
- t(8;14) translocation
- c-myc oncogene overexpression
- Rapidly responsive to Tx
What is seen on the histology of Burkitt’s lymphoma?
STARRY SKY APPEARANCE
What is the general management of Burkitt’s lymphoma?
Chemotherapy:
- Rituximab (anti-CD20 found on B cells)
+ Secondary CNS prophylaxis
What are some features of endemic Burkitt’s lymphoma?
- Most common malignancy in equatorial Africa
- EBV-ASSOCIATED
- JAW INVOLVEMENT + abdominal masses
What are some features of Sporadic Burkitt’s lymphoma?
- Foun doutside Africa
- EBV-ASSOCIATED
- Jaw less commonly involved
What are some features of immunodeficiency Burkitt’s lymphoma?
- Non-EBV associated
- HIV/post-transplant pts
What are some features of Diffuse Large B Cell Lymphoma?
- Middle aged + elderly
- Aggressive
- Can be transformed from low grade lymphoma
What is seen on histology of Diffuse Large B cell lymphoma?
Sheets of large lymphoid cells
What is the treatment of Diffuse Large B Cell lymphoma?
- Rituximab-CHOP
- Auto-SCT/CAR-T for relapse
What are some features of Mantle Cell lymphoma?
- Middle-aged
- M>F
- AGGRESSIVE
- Disseminated at presentation
- Median survival = 3-5yrs
- t(11;14) translocation = over-expression of cyclin D1 (causes deregulation)
What is seen on histology of Mantle Cell lymphoma?
Angular/CLEFTED nuclei
What is the treatment of Mantle Cell Lymphoma?
- Rituximab-CHOP + High-dose Cytarabine
- Auto-SCT for relapse
What are some features of Follicular lymphoma?
- Indolent
- Mostly incurable
- Median survival = 12-15yrs
- t(14;18) translocation = fusion of BCL2 gene
What is seen on histology of follicular lymphoma?
- Follicular pattern
- Nodular appearance
What is the treatment of follicular lymphoma?
- Watch + wait
- Rituximab or obinutuzumab + chemo
What are some features of MALT (Mucosal assocaited lymphoid tissue)?
- Marginal zone NHL
- Middle-aged
- Chronic antigen stimulation: H. pylori causing gastric MALT OR Sjogren’s causing parotid lymphoma
What is the treatment of MALT?
- REMOVE ANTIGENIC STIMULUS
(e.g. H. pylori) - Triple therapy
- Chemotherapy
What are some different T-cell lymphomas and some comments?
Anaplastic Large Cell Lymphoma:
- Children + young adults
- Aggressive
- Large “epithelioid” lymphocytes
- t(2;5)
- Alk-1 protein expression
Peripheral T-cell Lymphoma:
- Middle-aged + elderly
- Aggressive
- Large T cells
Adult T cell leukaemia/lymphoma:
- Caribbean + Japanese
- HTLV-1 infection
- Aggressive
- Flower Cells on blood film
Enteropathy-associated T cell lymphoma (EATL):
- A/w: longstanding coeliac disease
Cutaenous T cell lymphoma:
- A/w: mycosis fungoides (weird rashes)
What is the general treatment for T-cell lymphomas?
Alemtuzumab (anti-CD52)
