ALL

Question 1

What is the epidemiology of ALL and aetiology/risk factors?

Answer 1

Mostly affects under 4s

Genetics and Environment (e.g viruses)

Common in Downs syndrome

Question 2

What are the most common presenting symptoms of ALL?

Answer 2

Bone pain
Lymphadenopathy
Symptoms of pancytopenia

Question 3

What are some genetic mutations in ALL?

Answer 3

Philadelphia chromosome t(9:22)

Question 4

What are the different subtypes of ALL and what is the most common?

Answer 4

Common ALL (75%): CD10 present, pre-B phenotype

T-cell ALL (20%): usually in adolescent males with mediastinal mass

B-cell ALL (5%)

B cell is most common!!!!!!!!

Question 5

What do you see on a blood smear with ALL?

Answer 5

Blast cells

Question 6

What are poor prognostic factors with ALL?

Answer 6

Typical cure rate 70 to 90%

Age (<2 or >10)

Male

Non-caucasian

Performance status > 1

White cell count > 20 at diagnosis

Cytogenetics
t(9;22) - Philadelphia chromosome
t(4;11)

CNS involvement

Question 7

How is ALL managed?

Answer 7

Chemotherapy +/- Bone Marrow transplant

Refer to specialist centre and enrol in clinical trials
Pre-phase and supportive therapy

Pre-phase therapy: commenced on steroids with allopurinol and IV hydration to reduce risk of TLS
Leucopheresis: helps to mitigate the risk of TLS.
Supportive therapy: anaemia and thrombocytopenia may require treatment if severe. G-CSF may be given.
Induction chemotherapy

Those with CNS disease require intrathecal chemotherapy and prophylactic therapy may be used in those without to reduce the risk of CNS relapse

Maintenance therapy

Daily 6-mercaptopurine and weekly methotrexate

Stem cell transplant

Allogeneic stem-cell transplant may be considered. Reduces risk of relapse

Question 8

What are some of the complications of ALL?

Answer 8

Tumour lysis syndrome

Neutropenic sepsis

SVCO

Chemotherapy side-effects: early (e.g. mucositis, nausea and vomiting, hair loss) or late (e.g. cardiomyopathy, secondary malignancies)