Acute lymphoblastic leukemia

Question 1

acute lymphoblastic leukemia- what?

Answer 1

Malignant disease of primitive lymphoid cells (lymphoblasts)

Leukemia= cancer of particular line of stem cells in the bone marrow

Acute= rapidly progressive

Uncontrolled growth of immature BLAST CELLS i.e. monoclonal haemopoiesis

Question 2

acute lymphoblastic leukemia- who?

Answer 2

Most common childhood cancer (peaks around 2-4 years)
Can also affect adults over 45

Often associated with Down syndrome

Associated with the Philadelphia chromosome (t(9:22) translocation) in 30% of adults and 3-5% of children with Acute lymphoblastic leukemia

Question 3

acute lymphoblastic leukemia- presentation?

Answer 3

MARROW FAILURE:
* Anaemia
* Infections
* Bleeding

INFILTRATION:
* Hepatosplenomegaly
* Lymphadenopathy
* Extramedullary area involvement e.g. CNS and testis (doesn’t happen in AML)
Bone pain

Question 4

acute lymphoblastic leukemia- blood findings?

Answer 4

FBC:
* Very high WCC
* Pancytopenia is possible

Blood film: presence of BLASTS
* Large size
* High nuclear: cytoplasm ratio
* Prominent nucleolus

By definition an acute leukaemia involves an excess of ‘blasts’ (>/=20) in either the peripheral blood or bone marrow

Question 5

describe what a blast cell looks like

Answer 5

Large size
High nuclear: cytoplasm ratio
Prominent nucleolus

Question 6

investigations- acute lymphoblastic leukemia

Answer 6

FBC:
* Very high WCC
* Pancytopenia is possible

Blood film: presence of BLASTS
* Large size
* High nuclear: cytoplasm ratio
* Prominent nucleolus

By definition an acute leukaemia involves an excess of ‘blasts’ (>/=20) in either the peripheral blood or bone marrow

Coagulation screen

Bone marrow aspirate:
* Morphology
* Immunophenotype
* Cyto/ molecular genetics- diagnostic utility, prognostic significance
Trephine- enable better assessment of cellularity and is helpful when aspirate is sub optimal

Question 7

acute lymphoblastic leukemia- treatment?

Answer 7

70-90% cure rate in kids

SUPPORTIVE- blood/ platelet infusion

Chemo (takes 2-3 years)
Hickman line to make things easier + provide long term central venous access

Question 8

SE of chemo?

Answer 8

• Tumour lysis syndrome ( presents as metabolic electrolyte abnormalities)
  
  • N+V
  • Hair loss
  • Infection (bacterial, fungal, protozoal)

Late effect: loss of fertility, cardiomyopathy with anthracyclines

Question 9

treatment tumour lysis syndrome?

Answer 9

allopurinol

Question 10

what is used to make chemo easier + provide long term central venous access?

Answer 10

Hickman line

Question 11

patient with acute lymphoblastic leukemia comes in and has neutropenic fever- treatment?

Answer 11

Anti infection- more susceptible to bacterial + fungal infection
-As soon as neutropenic fever give empirical broad spectrum antibiotics
-If unresponsive may be fungal