Leukaemia Flashcards
What is an acute leukaemia?
Rapidly progressive clonal malignancy of the marrow/blood with maturation defects
Acute leukaemia is defined as an excess of what?
‘Blasts’, by > 20% in the peripheral blood or bone marrow
Acute leukaemias will cause a decrease/loss of normal haemopoietic reserve. What clinical features will this cause?
Anaemia, thrombocytopenia, neutropenia
What are the two main subtypes of acute leukaemia?
Acute myeloid leukaemia and acute lymphoblastic leukaemia
ALL is a disease of what?
Primitive lymphoid cells, i.e. lymphoblasts
What is the most common childhood cancer?
Acute lymphoblastic leukaemia
What are the 4 main presenting features of acute lymphoblastic leukaemia?
Marrow failure, leukaemic effects, infiltration (hepatosplenomegaly and superficial lymphadenopathy) and bone pain
ALL can present with ‘leukaemic effects’ - what is meant by this?
There are high counts of leukaemic cells in the blood. This can cause obstruction of circulation, and also involvement of areas outside the marrow and blood such as the CNS or testis
Acute myeloid leukaemia is more common in who?
Elderly (> 60)
How can acute myeloid leukaemia arise?
De novo, or secondary to an underlying haematological disorder
What is the presentation of AML?
Marrow failure
Subgroups of AML can have characteristic presentations. What are some examples of these?
DIC or gum infiltration
What are the 3 main investigations for acute leukaemia?
Blood count and film, coagulation screen, bone marrow aspirate
In acute leukaemia, what will a blood count and film show?
Reduction in normal, presence of abnormal, lots of large primitive cells
Auer Rod cells are seen in what type of malignancy?
Acute myeloid leukaemia
How can ‘blasts’ be identified on a blood film?
High nuclear: cytoplasmic ratio
What are the main things to do with a bone marrow aspirate for acute leukaemia?
Morphology and immunophenotyping
What investigation is required for a definitive diagnosis of acute leukaemia?
Immunophenotyping from a bone marrow aspirate
What is the purpose of cyto/molecular genetics in the diagnosis of acute leukaemia?
Prognostic significance
When is a trephine (piece of bone) used to help diagnose acute leukaemia?
If the aspirate is sub-optimal
What is the main treatment of acute leukaemias?
Multi agent chemotherapy
How is chemotherapy performed for ALL?
Can last 2-3 years with different phases of varying intensity
How is chemotherapy performed for AML?
Intensive - 2-4 cycles of 5-10 days of chemo followed by 2-4 weeks recovery
What are the main problems of marrow suppression in terms of treatment for acute leukaemia?
Anaemia, neutropenia (infections) and thrombocytopenia (bleeding)
What type of organisms can cause fulminant life-threatening sepsis in neutropenic patients?
Gram -
Apart from marrow suppression, what are some complications of treatment for acute leukaemia?
N+V, hair loss, liver/renal dysfunction, tumour lysis syndrome, infections
What are some late effects of treatment for acute leukaemia?
Loss of fertility and cardiomyopathy with anthracyclines
If there is a neutropenic fever in a patient receiving treatment for acute leukaemia, what is the management?
Empirical treatment with broad spectrum antibiotics, particularly covering gram - organisms
What may you suspect if there has been prolonged neutropenia and persistent fever unresponsive to antibiotics?
Fungal infection
What are the outcomes of acute myeloid leukaemia?
Many patients will go into remission, but many will relapse
Apart from chemotherapy, what are some other treatment options for acute myeloid leukaemia?
Targeted treatments and allogenic stem cell transplantation
Molecular targeting with kinase inhibitors can be used as a treatment in what?
ALL with the Philadelphia chromosome
What are the outcomes of acute lymphoblastic leukaemia?
70-90% cure rate with chemotherapy in children
What is the pathology behind chronic myeloid leukaemia?
Uncontrolled proliferation of myeloid cells (granulocytes and their precursors, other lineages e.g. platelets)
Stem cell transplants are not usually done in who?
Patients aged > 60
In acute leukaemia, the cells do not mature. What happens in chronic leukaemia?
The cells mature, but there are too many of them
Describe the onset of clinical features in chronic myeloid leukaemia?
Mostly chronic and insidious
How is chronic myeloid leukaemia often picked up?
On routine bloods (the patient is asymptomatic)
What are some clinical features of chronic myeloid leukaemia?
Splenomegaly, hypermetabolic features (weight loss, fever, sweats), gout, priapism
Who does chronic myeloid leukaemia tend to occur in?
People aged 40-60, with a male predominance
What happens to the blood count in chronic myeloid leukaemia?
Reduced/normal Hb, leucocytosis (neutrophilia, eosinophilia, basophilia), thrombocytosis
What is a bone marrow biopsy used for in chronic myeloid leukaemia?
To check for the presence of ‘blasts’ which would be suggestive of AML
What is the hallmark of chronic myeloid leukaemia?
The Philadelphia chromosome
What change occurs to form the Philadelphia chromosome?
A translocation between chromosomes 9 and 22, resulting in a new gene known as BCR-ABL1
The Philadelphia chromosome causes the formation of the BCR-ABL1 gene - what is the product of this?
A tyrosine kinase
What is the treatment for patients with chronic myeloid leukaemia? What type of drug is this?
Imatinib / tyrosine kinase inhibitor
What is the outcome for patients with chronic myeloid leukaemia?
Normal life expectancy with imatinib treatment
