Haem

Question 1

what genetic condition is associated with ALL?

Answer 1

downs syndrome

Question 2

what clinical signs may be present during ALL?

Answer 2

lymphadenopathy

HSM

Question 3

what are DD for ALL?

Answer 3

bruising (immune thrombocytopenia)
recurrent infections (immune deficiency)
lymphadenopathy 
CNS symptoms 
aplastic anaemia

Question 4

what fluid requirements will a child with ALL need?

Answer 4

will require hyperhydration, to prevent hyper viscosity due to high WBC count

Question 5

how long is maintenance treatment required for girls and boys?

Answer 5

Maintenance treatment is 2 years for girls and 3 years for boys.

Question 6

state some complications of ALL?

Answer 6

Infertility, avascular necrosis, peripheral neuropathy and anxiety

Question 7

what virus is associated with lymphoma?

Answer 7

Epstein-Barr Virus

Question 8

what medication can be used for tumour lysis syndrome?

Answer 8

Allopurinol or rasburicase

hyper hydration is also required

Question 9

what is tumour lysis syndrome ?

Answer 9

rapid lysis of tumour cells causes release of large amounts of phosphorus, potassium and calcium leading to potential kidney damage. This is most likely to occur when chemotherapy is first commenced

Question 10

what is used to manage a potentially compromised airway from mediastinal masses?

Answer 10

airway support and high dose steroids

Question 11

what is the name of Nephroblastoma?

Answer 11

Wilms tumour

Question 12

what age is most affected by Nephroblastoma?

Answer 12

3 and a half years old

Question 13

what type of genes are associated with Nephroblastoma?

Answer 13

tumour suppressor genes

Question 14

state three syndromes that have an increased risk of Nephroblastoma?

Answer 14

WAGR
Denys-drash
Beck with-Wiedemann

Question 15

what does WAGR syndrome stand for?

Answer 15

Wilm’s tumour, Aniridia, Genitourinary malformations, and Retardation

Question 16

what will be found on examination for Wilms tumour?

Answer 16

abdo distension
unilateral or bilateral palpable renal masses
HTN

Question 17

what are DD for Wilms tumour?

Answer 17

Polycystic kidney disease
hydronephrosis
neuroblastoma

Question 18

what would neuroblastoma present with?

Answer 18

HTN, abdo pain, fever

Question 19

what clinical signs would be seen with neuroblastoma?

Answer 19

periorbital ecchymosis

abdominal mass which crosses the midline

Question 20

what are the 5 stages of nephroblastoma?

Answer 20

1- confined to kidney 
2- spread beyond the kidney 
3- spread to lymph nodes 
4- distant mets 
5- bilateral tumours

Question 21

what stages can be treated with surgery (nephrectomy) ?

Answer 21

stage 1 and 2

Question 22

What percentage of childhood cancers does nephroblastoma account for?

Answer 22

5%

Question 23

What is the most common malignant differential diagnosis of nephroblasoma of an abdominal mass in a pre-school child?

Answer 23

neuroblastoma

Question 24

What initial imaging test is done when an abdominal mass is present?

Answer 24

US

Question 25

What is the initial management of nephroblastoma?

Answer 25

supportive

Question 26

Where do Wilms tumours most commonly metastasise to?

Answer 26

lung

Question 27

What percentage of patients with metastatic disease of nephroblastoma are expected to be cured?

Answer 27

85%