Acute lymphoblastic leukaemia

Question 1

What is ALL

Answer 1

A malignant disorder of the bone marrow

Commonest cancer of the paediatric population

Question 2

When does incidence of ALL peak?

Answer 2

2-5years

Question 3

Which sex is more affected by ALL

Answer 3

Males

Question 4

Describe the cause of ALL

Answer 4

Multifactorial
Infection
Genetics
Environmental exposures

Question 5

Describe the pathophysiology of ALL

Answer 5

Disruption in the regulation and proliferation of lymphoid precursor cells in the bone marrow leads to excessive production of immature blast cells and a subsequent drop in the number of functional red, white blood cells and platelets

Question 6

Which genetic condition is known to increase the risk of ALL?

Answer 6

Trisomy 21 - Downs

Question 7

Describe the clinical features of ALL

Answer 7

Anaemia - lethargy and pale

Thrombocytopenia - easy bruising/bleeding

Leukopenia - fever and infections

Bone pain - increased pressure in the hyperplastic marrow

Weight loss

Malaise

CNS involvement - headaches and seizures

Question 8

What examination findings will there be for a child with ALL?

Answer 8

Pale
Unexplained/excessive bruising
Lymphadenopathy
Hepatosplenomegaly

Question 9

List some differentials of ALL

Answer 9

Immune thrombocytopenia

Trauma

Non accidental injury

Immune deficiency

Reactive lymphadenopathy

Raised ICP

CNS infection

Aplastic anaemia

Question 10

What investigations are necessary for ALL

Answer 10

FBC - pancytopenia or anaemia and thrombocytopenia with significant lymphocytosis
Blood film - blasts
CXR
Bone marrow aspirate/trephine
Lumbar puncture

Question 11

List some factors associated with a poor prognosis

Answer 11

WCC >50 at presentation 
Male 
Presence of blasts in the CNS
Morphology and cytology 
Age<1yo/10yo

Question 12

List some factors associated with a better prognosis of ALL

Answer 12

Female
Age 1-10 at presentation
Morphology and cytology

Question 13

What is the immediate management of ALL

Answer 13

Resuscitate and stabilise the unwell child

Any child with high WCC needs hyperhydration to prevent hyperviscotisty

Steroids if mediastinal mass

Broad spectrum antibiotics if infection

Question 14

Which protocol are the majority of ALL patients managed with?

Answer 14

UKALL 2011 protocol

Question 15

What is the treatment for ALL?

Answer 15

Chemotherapy

Supportive treatment - blood products and prophylactic anti-fungal treatment

Question 16

How is chemotherapy for ALL given?

Answer 16

Intravascularly
Orally
Intrathecally

Question 17

What does intrathecally mean?

Answer 17

Into the CSF

Question 18

How long does maintenance treatment last for boys and girls?

Answer 18

Boys - 3 years

Girls - 2 years

Question 19

What percentage of children with ALL are expected to survive?

Answer 19

90%

Question 20

Describe the follow up of ALL children

Answer 20

All those treated for ALL should have regular follow up and assessment for 5 years after treatment is completed to assess for any signs of relapse and then yearly review thereafter to evaluate for late effects of chemo

Question 21

List some complications of the chemo for ALL

Answer 21

Infertility
Avascular necrosis
Peripheral neuropathy
Anxiety