Lymphoma 1: MDT Flashcards
Define lymphoma
Lymphoma means neoplastic (malignant) tumour of lymphoid cells
Where are lymphomas most commonly found?
- Lymph nodes, bone marrow and/or blood (the lymphatic system)
- Lymphoid organs: spleen or the gut-associated lymphoid tissue
- Skin (often T cell disease)
- Rarely “anywhere” (CNS, occular, testes, breasts etc)
What is the incidence of lymphoma?
200 new cases per year for every million of the population
- Non-Hodgkin’s lymphoma = 80%
- Hodgkin Lymphoma = 20%
How many types of lymphoma are there?
>60 types of lymphoma exist
What are the downside of an adaptive immune system that increases lymphoma risk?
- DNA molecules are
- cut and rejoined plus
- undergo deliberate point mutation, which allows for the generation of immunoglobulin and T cell receptor diversity
- Dependent on apoptosis (90% of normal lymphocytes die in the germinal center)
- Ensures antibody specificitty - preventing autoimmune disease
- Apoptosis is switched off in germinal center (cancer)
- Acquired DNA mutation in pro-apoptotic genes (cancer)
- Rapid cell proliferation in the germinal center
- Cell division = risk of DNA replication error
Describe lymphoma recombination associated translocations
- Involve the Ig locus
- Ig promoter highly active in B cells
- Bring intact oncogenese close to the Ig promoter
- Oncogenes may be anti-apototic, proliferative
- bcl2
- bcl6
- Myc
- cyclin D1
What are the known risk factors for lymphoma?
- Constant antigenic stimulation
- Infection (direct viral infection of lymphoyctes)
- Loss of T cell function
Describe lymphoma and chronic antigenic stimulation, and what conditions could be a risk
- Initially antigen dependent, eventually become autonomous (malignant)
Conditions:
- H.pylori: Gastric MALT area affected
- Sjorgen syndrome: marginal zone NHL of parotid lymphoma
- Coeliac disease: small bowel T cell lymphoma (enteropathy associated T cell Non-Hodgkin Lymphoma (EATL))
Describe viral infection and the risk of lymphoma:
- Direct Viral integration
- EBC infection and immunosuppression
- Direct viral integration
- HTLV1 infects T cells by vertical transmission
- Caribbean and Japan carriers
- May develop Adult T cell leukemia (2.5% at 70 years)
- EBV infection and immunosuppression
- EBV infects B lymphocytes
- Healthy carrier state maintained by cytotoxic T cells kill EBV antigen expressing B cells
- Loss of T cells function give risk of EBV driven lymphomas
HIV - 60 fold increase in lymphoma (high grade B-Non-Hodgkin’s lymphoma)
Post transplant lymphoproliferative disorder
Describe what is involved in the diagnosis and staging of lymphoma
- Histological diagnosis
- Anatomical stage - using CT PET, BM biopsy etc
- Prognostic factors
- LDH
- Beta2 microglobulin
- Albumin
- Kidney/BM function
- What do Reed Sternberg cells signify?
- Classical Hodgkin Lymphoma
15% of all lymphoma/leukemia
15% of lymphoma is classical hodgkin lymphoma, describe what makes up the other 85%
Other 85% of lymphoma is Non-Hodgkin lymphoma
B cell
- Precursor B lymphoblastic leukemia (B-ALL) or lymphoma
- Mature B cell neoplasm - DLBCL, Follicular NHL, CLL etc
T or NK cell
- Precursor T lymphoblastic leukemia or lymphoma (T-ALL)
- Mature T and NK neoplasm - PTCL, Anaplastic, cutaneous
- Describe the epidemiology of Hodgkin Lymphoma
- What are the signs and symptoms of Hodgkin lymphoma
- 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
- Signs and Symptoms:
- Painless enlargement of lymph node/nodes which may cause obstructive symptoms/signs
- Constitutional symptoms:
- Fever
- Night sweats
- Weight loss (the B symptoms)
- Pruritis
- RARE: Alcohol induced pain
Describe the sub-types of Classical Hodgkin Lymphoma
- Nodular sclerosing (80%) - good prognosis
- Mixed cellularity (17%) Good prognosis
- Lymphocyte rich (rare) Good prognosis
- Lymphocyte depleted (rare) Poor prognosis
Nodular lymphocyte predominant HL 5% - More a disorder of the elderly
- How is Hodgkin Lymphoma staged?
- What is the staging system?
1.
- Pathological diagnosis of a lymph node biopsy
- FDG-PET/CT scan
- Consider biospy of other site if possiblly infiltrated
- Stages
- I = one group of nodes
- II = >1 group of nodes same side of the body
- III = nodes above and below the diaphragm
- IV = extra-nodal spread
- Suffix A if none of the below, B if any of the below:
- Fever
- Unexplained weight loss >10% in 6 months
- Night sweats