Haemato-oncology 2 Flashcards
• What is a leukaemia?
Clonal proliferation of malignant blood cells
• What is an acute leukaemia?
A leukaemia of the immature precursor cells (a.k.a blast cells).
• How do acute and chronic leukaemias compare?
Cells involved
Onset & Progression
Lethality
Acute leukaemia
Blasts
Rapid
Will kill you in 2-8 weeks (sometimes even the day you present)
Chronic leukaemia
More mature cells
Slower
Can transform to acute leukaemia and kill you, otherwise are more indolent
What chromosomal abnormality predisposes you to both AML and ALL?
Down’s syndrome
• Which acute leukaemia is more common?
AML
Acute Myeloid Leukaemia (AML)
o More common as you get older?
• How do you diagnose AML?
Flow cytometry - what CD antigens are expressed in AML?
Yes
FBC
Blood film
Bone marrow histology (blasts MUST be >20% of nucleated cells)
CD13+ and CD33+ are expressed in AML
• What is APL? (Acute promyelocytic leukaemia)
what translocation?
what mutation?
APL is a medical emergency, what do you do about it?
a specific subtype of AML that is caused by a translocation between chromosomes 15 and 17, resulting in a PML-RARα fusion gene
is associated with coagulation problems → bleeding
give all-trans retinoic acid immediately and for 3 months thereafter, which forces APL cells to differentiate and prevents proliferation
• What is the most important prognostic indicators in AML?
Age?
Genotype?
Cytogenetics - translocations or chromosome abnormalities good or bad?
younger patients do better
patients who are FLT3-negative and NPM1-positive do better
Chromosome abnormalities are bad translocations are good
treatment strategies for AML:
if bad prognosis?
in all patients - give combination chemotherapy which leads to?
- therefore you support patient by giving?
bone marrow transplant
prolonged pancytopenia
transfusion of red cells and platelets
prompt Abx for any infections
Acute Lymphoid Leukaemia (ALL)
o Peak age ?
what clinical signs are different to AML?
Diagnosis is same as AML except in flow cytometry which CD antigens are there?
Tx?
Prognosis in:
Children?
Adults?
- 2-10 yr olds and elderly
- CNS disease and bone pain
- CD10, CD19, CD22
- tx = multidrug chemotherapy -
children = 50% cure rate adults = 85% cure rate
• What is minimal residual disease, MRD?
name given to small numbers of leukaemic cells that remain in the patient during remission. These cells can cause relapse.
• What techniques are used to try and detect the MRD in a patient in remission?
- Microscopy of a blood film
- FISH
- Immunophenotyping
- PCR
which one is most sensitive for detecting low number of cells?
PCR (increasing sensitivity from 1-4)
• So, what is complete remission?
When you get the MRD so low that even the most sensitive PCR testing can’t pick up any cancer cells (hence they are MRD negative). This doesn’t mean the person doesn’t have ANY cells left (they may have 100-1000 cells left, but we cant detect them).
what is the mortality rate with bone marrow transplants?
Why is there such a high mortality?
20%
- Neutropenic sepsis – once you give the initial chemotherapy and before the new transplanted cells proliferate in the bone marrow, the person is extremely vulnerable to infections because you’ve wiped out their whole immune system. Note that 1/3rd of people with neutropenic sepsis DO NOT have a fever
- Graft versus host disease – the new immune system from the transplanted cells start attacking the patient’s cells as it sees it as foreign
• What long term consequences are there of BMT?
infertility
secondary cancers
