Lymphoproliferative Disorders Flashcards
How is the diagnosis of leukaemia or lymphoma made?
Defined by malignant cell characteristics:
- Diagnosis made by biopsy (of lymph node or bone marrow etc)
How is staging done?
- Clinical examination
- CT scan
What does staging describe?
- Location and extent of disease
- Info about prognosis
- Influences treatment
How is lymphoma classified?
Hodgkin lymphoma is specific disease, non-Hodgkin lymphoma is everything else (more than 70 conditions)
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What are the main lymphoproliferative disorders?
- Acute lymphoblastic leukaemia (ALL)
- Chronic lymphoblastic leukaemia (CLL)
- Hodgkin lymphoma
- Non-Hodgkin lymphoma (NHL)
- High grade (diffuse large B-cell lymphoma)
- Low grade (follicular, marginal zone)
What does ALL stand for?
- Acute lymphoblastic leukaemia (ALL)
What does CLL stand for?
- Chronic lymphoblastic leukaemia (CLL)
What does HL stand for?
Hodgkin lymphoma
What does NHL stand for?
- Non-Hodgkin lymphoma (NHL)
What is the most common lymphoproliferative disorder?
Most common is high grade NHL
ALL is a disorder of what cells?
Cancerous disorder of lymphoid progenitor cells
Describe the pathophysiology of ALL?
- Normally immature cells that rapidly proliferate and differentiate into lymphocytes
- In leukaemia, no differentiation and instead rapid and uncontrolled growth and accumulation
- Usually in bone marrow but they can go anywhere
Describe the epidemiology of ALL?
(incidence, age)
- Incidence 1-2/100,000
- 75% cases children <6 years
Describe the presentation of ALL?
- 75-90% cases are B-cell linage
- 2-3 week history of bone marrow failure or bone/joint pain
- Weight loss
- Anaemic symptoms
Describe the investigations for ALL?
- Blood count
- Low haemoglobin
- High WCC
- Low platelet
- Bone marrow aspirate
- Full of B lymphoblasts
- Blood film
- Large cells
- Immunophenotyping
- Used to tell what markers cells carry
- They express CD19 – all B cells have this
- And express CD34, TDT – markers of very early, immature cells
What is seen in the FBC for ALL?
- Low haemoglobin
- High WCC
- Low platelet
What is seen in the bone marrow aspirate for ALL?
- Full of B lymphoblasts
What is immunophenotyping used for?
- Used to tell what markers cells carry
- They express CD19 – all B cells have this
- And express CD34, TDT – markers of very early, immature cells
Describe the treatment of ALL?
- Standard treatment - chemotherapy
- Induction chemotherapy to obtain remission
- Consolidation therapy
- CNS directed treatment
- Maintenance treatment for 18 months
- Stem cell transplantation (if high risk)
- Newer therapies
- Bi-specific T-cell engagers
- Drug – Blinatumumab
- CAR-T cell therapy
- Modify patients own T cells to express receptor for CD19 marker
- Side effects – cytokine release syndrome (fever, hypotension, dyspnoea), neurotoxicity (confusion, seizure, headache, focal neurology, coma)
- Bi-specific T-cell engagers