Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Pt.2 Flashcards
Describe the typical presenting features of enteropathy-associated T cell lymphoma.
- Abdominal pain/ obstruction/ bleeding/ perforation
- Systemic symptoms
Why is it important to prevent EATL by following a strict gluten-free diet?
EATL responds poorly to chemotherapy and is usually fatal
What is the most common leukaemia in the Western world and what is its median age of presentation
Chronic lymphocytic leukaemia - proliferation of mature B-cells
Median age of presentation is 72
What are the typical laboratory findings in a patient with CLL?
- Lymphocytosis
- Smear cells
- Normocytic normochromic anaemia
- Thrombocytopaenia
- Bone marrow lymphocytic replacement of normal marrow elements
NOTE: it is indolent so is often only picked up on routine blood tests
What distinctive antigen phenotype (presence and absence) is suggestive of:
- Mature B cells
- Mature T cells
- Mature B cells:
- CD19 positive
- CD5 negative
- Mature T cells:
- CD19 negative
- CD5 positive
- CD3 positive
- CD4 or CD8 positive
Which antigen phenotype is suggestive of CLL?
CD19+ and CD5+
NOTE: this could potentially also be mantle cell lymphoma
Which staging system is used for CLL?
Rai and Binet
Binet: stages A-C depending on number of lymphoid areas (< or > 3, Hb and platelets)
Which specific tests are used in CLL to help gauge prognosis?
FISH cytogenetic panel
- TP53 mutation status
IgH V gene mutation status
CD38 expression (associated with poor prognosis)
How do TP53 and IgH V gene mutations affect prognosis
TP53 - mutation/deletion associated with worse prognosis
IgH V gene - mutation associated with better prognosis
What is the difference between the VH genes of pre- and post-germinal centre B cells?
Pre-germinal centre: variable region is unmutated and looks identical to germline
Post-germinal centre: undergone somatic hypermutation so variable region is mutated and looks different to germline
Describe the immunoglobulin levels you would expect to see in CLL.
Hypogammaglobulinaemia - because the malignant B cells are suppressing antibody production by other B cells
Leads to increased risk of infection
What is the term used to describe CLL changing into a high grade lymphoma?
Richter transformation - 1% risk per year
What are some supportive measures used in the treatment of CLL?
- Vaccination (covid, flu, pneumococcus)
- Infection prophylaxis and treatment (e.g. PCP prophylaxis)
How would autoimmune cytopaenias caused by CLL be treated?
Steroids
NOTE: 2nd line is rituximab
How would a Richter transformation be treated?
R-CHOP
