Lymphoma 2 Flashcards
What is the presentation of lymphoma?
- Painless progressive lymphadenopathy
- Palpable node
- Extrinsic compression of any “tube”
- Eg Ureter, Bile duct, large blood vessel, bowel, trachea, oesophagus
- Infiltrate/impair an organ system
- E.g. skin rash, ocular&CNS, liver failure
- Recurrent infections
- Constitutional symptoms
- Coincidental e.g. FBC, Imaging
What is the presentation of non-Hodgkin Lymphoma. What investigation would you do
- Lymphadenopathy
- Biopsy
What is this image indicative of?
Left to rightÑ Mesenteric and axillary, axillary and cervical, splenomegaly → non-bodkin lymphoma
How do we diagnose and stage lymphoma? What does is dictate?
What types of lymphoid malignancies exist? How prevalent are they?
- 15% Lymphoma
- Reed Stemberg cells
- Classical Hodgkin Lymphoma (cGL) (or NLPHL)
- 85% Non Hodkin Lymphoma
- B cell
- Precursor B lymphoblastic leukaemia (B-ALL) or lymphoma
- Mature B cell neoplasm DLBCL, Follicular NHL, CLL etc
- T or NK cell
- Precursor T lymphoblastic leukaemia or lymphoma (T-ALL)
- Mature T and NK neoplasm PTCL, Anaplastic, Cutaneous
- B cell
Describe the epidemiology of Hodgkin Lymphoma.
- 1% of all cancer, 3:100,000 population
- HL is more common in males than females.
- Bimodal age incidence
- Most common age 20-29, young women NS subtype
- Second smaller peak affecting elderly >60 years old
How does Hodgkin Lymphoma present?
- Painless enlargement of lymph node/nodes.
- May cause obstructive symptoms/signs
- Constitutional symptoms; (the B symptoms 1)fever, 2) night sweats 3) weight loss . Pruritis and rarely alcohol induced pain
How do we classify HL?
- Classical HL
- Nodular sclerosing 80% Good prognosis (causes the peak incidence in young women)
- Mixed cellularity 17% Good prognosis
- Lymphocyte rich (rare) Good prognosis
- Lymphocyte depleted (rare) Poor Prognosis
- Nodular Lymphocyte predominant HL 5% (disorder of the elderly multiple recurrences)
How do we stage HL?
- Staging: Following pathological diagnosis of a lymph node biopsy patients are ‘staged’ this has prognostic significance and also may determine the best approach for therapy.
- FDG-PET/CT scan
- Consider biopsy of other site if possibly infiltrated e.g. liver
Stage
- I; one group of nodes
- II; >1 group of nodes same side of the diaphragm
- III; nodes above and below the diaphragm
- IV; extra nodal spread
- Suffix A if none of below, B if any of below
- Fever
- Unexplained Weight loss >10% in 6 months
- Night sweats
Who gets cHL Nodular sclerosing? How does it present?
cHL nodular sclerosing sub type
- Young women(>men) 20-29 years
- Neck nodes and mediastinal mass(may be massive and compress SVC or trachea)
- May have B symptoms
- Needs a Tissue diagnosis
What is ABVD?
ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- DTIC
- ABVD, is given at 4-weekly intervals.
- ABVD is Effective treatment
- Preserves fertility (unlike MOPP the original chemo)
- Can cause (long term)
- Pulmonary fibrosis
- cardiomyopathy
How do we treat cHL?
-
Chemotherapy (essential for cure)
- ABVD 2-6 cycles (depends:stage&interimresponse)
- PET CT
- Interim: After x2 cycles, response assessment
- End of Treatment: Guides need for additional radiotherapy
- +/- Radiotherapy
- Relapse {salvage chemotherapy}
- High dose chemotherapy + Autologous PB stem cell transplant as support
Describe the use of radiotherapy ion HL
What is the outcome of Hodgkin Lymphoma?
- Outcome of therapy
- Older patients generally do less well as do those with lymphocyte-depleted histology.
Prognosis:
Cure rate ranges from 50-90%.
- Over 80% of patients with stage I or II disease are cured
- Only 50% of stage IV patients are cured
- What does cure mean ?
- Overall 80% are long term survivors (can we improve)• 10% die from relapse of HL (first 10 years)
10% die from treatment complications (after 10 years)
•“Curing” cHL in a 25-year old woman using chemo plus radiotherapy does not guarantee long term survival !
Define Non Hodgkin Lymphoma. What is the incidence? What is the clinical course?
- Neoplastic proliferation of lymphoid cells.
- Incidence rising 200/million population/year
- Clinical course highly variable
- fastest proliferating malignancy (Burkitt Lymphoma)
- indolent diseases (eg Follicular NHL with a possible 25 yearsurvival)
- Antibiotic responsive disease such as Gastric MALT
Describe the pictures
Left to right:
- BL (endemic type)
- FL
- Reactive
- Folllicular lymphoma
How do we stage Non Hodgkin Lymphoma? What are the prognostic markers? What effect does this have?
Stage the disease (as in Hodgkin Lymphoma)
- CT scan
- PET scan (indicated in aggressive lymphomas)
- BM biopsy
- Lumbar puncture (if risk of CNS involvement )
Prognostic markers & Important tests
- LDH
- Performance status
- HIV serology (if appropriate HTLV1 serology)
- Hepatitis B serology (risk of reactivation if B cell depleting therapy given)
Plan Therapy (histology & Performance status)
- Urgent chemotherapy
- Monitor only {watch & wait} ?
- Antibiotic eradication (H.Pylori gastric MALT lymphoma) ?
What are the subtypes of Non Hodgkin Lymphoma?
What are the subtypes of Non Hodgkin Lymphoma?
What is the WHO classification of Non Hodgkin Lymphoma
Describe the NHL outlook of survival and response to chemotherapy.
Describe diffuse large B cell NHL.
How do we treat DLBCL? What is the prognosis?
Describe follicular NHL. What is it associated with? How do we treat them?
What are the initial therapeutic options for follicular NHL?
Describe the extra-nodal Marginal Zone Lymphomas (MZL). What is it associated with? Where is it only present?
What is enteropathy associated T cell lymphoma (EATL)? What is it associated with? What is the presentation of EATL?
Nodular Sclerosis
4
Describe the epidemiology of CLL?
- Proliferation of mature B-lymphocytes
- Commonest leukaemia in the western world
- Caucasian
- UK incidence 4.2/100,000/year
- Age at presentation median 72 (10% aged <55yrs)
- Relatives x7 increased incidence
What are the lab findings in CLL?
- Lymphocytosis between 5 and 300 x 109/l
- Smear cells
- Normocytic normochromic anaemia
- Thrombocytopenia
- Bone marrow Lymphocytic replacement of normal marrow elements
What does this image show?
CLL
What is the natural history of CLL?
How do we use immunophenotype in diagnosis?
What do CLL cells have?
CD5+ and CD19+
How can we determine CLL prognosis?
FISH = Fluorescent in-situ Hybridisation
If you lose TP53 - bad prognosis
What are the clinical issues in CLL?
What is CLL supportive care?
What are the Indications to treat CLL?
What are the tx options in CLL
What are the CLL targeted Rx
How does venetoclax work? What is the risk?
CD3- CD5- CD19+
IgHV unmutated and TP53 mut+
Block BCL2 protein
