Haem - Lymphoma 2 Flashcards
(22 cards)
Which Ig are associated with B cell lymphomas
Follicular NHL: IgH-BCL2
Mantle Cell lymphoma: IgH-Cyclin D1
Burkitt Lymphoma: IgH-MYC
What are the symptoms of lymphoma
Painless progressive lymphadenopathy (neck, axilla groin)
Compression symptoms
- Renal failure
- Obstructive jaundice
- Large blood vessels
- Bowel obstruction
- Trachea
- Oesophageal
Infiltration
- Brain: stroke, lOC, memory problems
- Skin rash: plaques, ulceration, nodules
- Ocular: blindess
- Liver: liver failure
Recurrent infections
B symptoms: fever, night sweats, weight loss
What investigations should be done for lymphoma
Bloods:
- LDH: raised
- Albumin
- U&Es
- HIV, hep B serology ± HTLV-1 (ensure hep B will not reactivate)
Anatomical stage
- bone marrow biopsy
- CT/PET
- Lumbar puncture if risk of CNS involvement
What is the epidemiology of Hodgkin’s lymphoma
1% of all cancer
Less common than non-Hodgkin’s
M > F
Bimodal age - 20-29yo (most common), >60yo (smaller peak)
What is the prognosis for classical HL
Nodular sclerosing 80%: Good prognosis (causes the peak incidence in young women) - thick bands of nodular tissue
Mixed cellularity 17%: Good prognosis
Lymphocyte rich (rare): Good prognosis
Lymphocyte depleted (rare): Poor Prognosis
Describe the features of nodular sclerosing HL sub-type (epidemiology, symptoms)
Seen in young women (F > M (20-29yo))
Neck nodes and a mediastinal mass; may have B symptoms
Mass may compress the SVC or trachea
Spreads contiguously
Describe the staging of lymphoma
Ann Arbor Staging
A = B symptoms absent
B = B symptoms present
E = involvement of single, contiguous, or proximal extranodal site
I: One node region involved on 1 side of the diaphragm
II: 2+ ipsilateral regions involved on 1 side of the diaphragm
III: Bilateral node involvement on both sides of the diaphragm
IV:
HL: Involvement of extranodal sites beyond those designated by ‘E’ (below)
NHL: Disseminated/multifocal involvement of ≥1 extralymphatic site OR isolated extralymphatic organ involvement with distant node involvement
Describe the treatment of Hodgkin’s lymphoma
Chemotherapy: ABVD (2-6 cycles, 4-weekly intervals) - adriamycin, bleomycin, vinblastine, DTIC (Dacarbazine)
± radiotherapy
Second line: salvage chemotherapy (high dose chemo + HSCT)
Third line: anti-CD30 (Brentuximab Vedotin)+ anti-PD1(nivolumab)
What is the prognosis for Hodgkin’s lymphoma
Elderly patients do worse than the worst-prognosis HL (lymphocyte-depleted type)
It is a highly curable disease, but the prognosis depends on stage
Over 80% in stage I or II disease are cured
50% of stage IV are cured
10% die from relapse within 10 years and 10% die from complications after 10 years
More likely to die of secondary malignancy of CV complications after 5 years
What is a common complication of Chemotherapy + radiotherapy for Hodgkin’s lymphom
Risk of damage to normal tissues (collateral damage)
Associated with increased risk of breast/lung/skin cancer, leukaemia/myelodysplasia
What are the prognostic markers for NHL
LDH (marker of cell turnover)
Performance status
HIV serology
Hep B serology (treatment for lymphoma may cause a dormant hep B to reactivated → fulminant liver failure)
What is the prognosis for NHL
Burkitt’s lymphoma – fastest growing human cancer
Follicular lymphoma (an indolent disease) – possible 25 year survival
HOWEVER, chance of being cured increases with more aggressive neoplasms
What is the management for NHL
Chemotherapy: R-CHOP (6-8 cycles) - rituximab, cylophosphamide, adriamycin, vincristine, prednisolone
Second line: autologous HSCT
Describe enteropathy associated T-cell lymphoma (cause and association, histology, presentation, response to treatment, prevention)
Caused by chronic antigen stimulation to gluten/gliadin → associated with coeliac disease
Histology: Mature T cells TdT, CD19
Presentation:
- Abdo pain, obstruction, perforation, GI bleeding
- Malabsorption
- Systemic symptoms
Responds poorly to chemotherapy, generally fatal
Can be prevented by strict adherence to a gluten-free diet
What phenotype is indicative of CLL
Normal T cells are CD5 positive, and CD 19 and 20 negative. While normal B cells are the other way around.
CLL B cells have an unusual phenotype (Monoclonal lymphocytosis with abnormal expression of CD5 = highly suggestive of CLL)
What is the phenotype of T cells
CD3 positive
CD4 or CD8 positive (Th-cell, CTL cell)
D19 NEGATIVE
CD5 POSITIVE
What is the prognosis for CLL
In a disorder of elderly
- 1/3 never progress
- 1/3 Progress, respond to CLL Rx (death from unrelated disorder)
- 1/3 Progress, require multiple lines of Rx, refractory disease, death from CLL
How is CLL staged
Rai and Binet staging
A: <3 lymphoid areas
B: >3 lymphoid areas
C Hb <100, Platelets <100
What are the prognostic lab factors for CLL
Cytogenetics (FISH panel)
Immunoglobulin gene (IgHV) mutation status
- IgH mutated
- IgH unmutated (Bad)
17p deletion
p53 muted
What are the complications of CLL
Non-functioning B cell production → hypogammaglobulinaemia → increased infection risk e.g. pneumonia, sinusitis
Metastasis to lymphoid organs e.g. lymph nodes, spleen
Bone marrow failure (anaemia, neutropenia thrombocytopenia)
Richter transformation → high grade lymphoma
Regulation of the surviving normal B cell population → autoimmune disease e.g. autoimmune haemolytic anaemia
What is the management for CLL
- Watch and wait
- Supportive treatment
- Vaccination: flu, pneumococcus (NOT live vaccines)
- Anti-infective prophylaxis and treatment - if young: allogenic HSCT
- If not, incurable by chemo → watch and wait
Richter transformation: high grade lymphoma R-CHOP
± new agents: ibrutinib (Bruton Tyroskine Kinase/BCR inhibitor), venetoclax (anti-Bcl2 oral agent), CAR-T (chimaeric antigen receptor T cells)
What are the poor prognostic features of CLL
Deletion of 13q (~133 months survival)
Trisomy 12 (~114)
Deletion of 11q (ATM) (~79)
Deletion of 17p (TP53) - loss of p53 tumour
