Oncology and haematology

Question 1

In what ethnicity is iron deficiency anaemia in children most common?

Answer 1

Those from the Indian subcontinent

Question 2

What is the daily iron requirement for children and toddlers?

Answer 2

High - 1mg/kg/day

Question 3

How is iron deficiency anaemia diagnosed in children?

Answer 3

Hypochromic microcytic anaemia + low serum ferritin

Question 4

How is iron deficiency anaemia managed in children?

Answer 4

Dietary advice + oral iron replacement therapy for 3/12

Question 5

What are the broad causes of anaemia?

Answer 5

1. Decreased/difficulty in red cell production
2. Increased red cell destruction
3. Combination of causes, e.g. anaemia of prematuirty
4. Blood loss (uncommon)

Question 6

What are the causes of decreased red cell production?

Answer 6

1. Iron deficiency anaemia

2. Red cell aplasia

Question 7

What are the different causes of red cell aplasia?

Answer 7

1. Congenital red cell aplasia
2. Transient erythroblastopenia of childhood (TEC)
3. Parvovirus B19 infection

Question 8

What are the causes of increased red cell destruction?

Answer 8

1. Hereditary spherocytosis
2. Thalassaemia
3. Isoimmune
4. Immune haemolytic anaemia
5. G6PD
6. Sickle cell disease

Question 9

How is sickle cell disease inherited?

Answer 9

Autosomal dominant

Question 10

What are the complications of sickle cell disease?

Answer 10

1. Bacterial infection
2. Acute chest syndrome
3. Priapism
4. Strokes

Question 11

How is G6PD inherited?

Answer 11

X-linked, therefore usually affects males, but can affect females

Question 12

What are the precipitants of haemolysis in G6PD?

Answer 12

1. Infection
2. Certain drugs
3. Fava beans (broad beans)
4. Naphthalene in mothballs

Question 13

How is hereditary haemolysis treated?

Answer 13

Folic acid + splenectomy if symptomatic

Question 14

How is hereditary haemolysis diagnosed?

Answer 14

Blood film

Question 15

How is hereditary haemolysis inherited?

Answer 15

Autosomal dominant, but in 25% of cases there is no FHx

Question 16

What are children with B-thalassaemia unable to produce?

Answer 16

HbA due to a mutation of the B-globulin gene

Question 17

How is B-thalassaemia treated?

Answer 17

Regular blood transfusion + prevention of iron-overload, with iron-chelation therapy with desferrioxamine or iron chelation

Question 18

What proportion of unilateral retinoblastomas are hereditary?

Answer 18

20%

Question 19

On what chromosome is the gene that predisposes a child to retinoblastoma?

Answer 19

Chromosome 13

Question 20

How do retinoblastoma present?

Answer 20

1. White reflex

2. Squint

Question 21

How is retinoblastoma managed?

Answer 21

Chemotherapy (for shrinkage) + laser treatment to the retina

Advanced cases = enucleation or radiotherapy

Question 22

What is another name for nephroblastoma?

Answer 22

Wilm’s tumour

Question 23

From where do Wilm’s tumours originate?

Answer 23

Embryonal renal tumour

Question 24

What is the typical presentation of a Wilm’s tumour?

Answer 24

Large abdominal mass in an otherwise well child

Question 25

How is a Wilm's tumour investigated?

Answer 25

USS or CT/MRU KUB

Question 26

How is a Wilm's tumour treated?

Answer 26

Initial chemotherapy + delayed nephrectomy | Radiotherapy in advanced disease

Question 27

From where do neuroblastomas arise?

Answer 27

From neural crest tissue in adrenal medulla and sympathetic nervous system - most commonly children present with an abdominal mass, but they can occur anywhere along the sympathetic chain from neck to pelvis

Question 28

What is the most common leukaemia in children?

Answer 28

Acute Lymphoblastic Leukaemia - 80% of cases

Question 29

How is ALL Dx?

Answer 29

FBC + bone marrow examination

Question 30

What are the causes of bruising when the platelet count is REDUCED?

Answer 30

1. Increased platelet destruction | 2. Impaired platelet production

Question 31

What are the causes of bruising when the platelet count is NORMAL?

Answer 31

1. Platelet dysfunction | 2. Vascular disorders

Question 32

What are the causes of platelet dysfunctions that can cause bruising?

Answer 32

CONGENITAL - rare disorders, e.g. Glanzmann thromboasthenia | ACQUIRED - uraemia; cardiopulmonary bypass

Question 33

What are the vascular disorders that can cause bruising?

Answer 33

CONGENITAL - Ehlers Danlos; Marfan's; HHT | ACQUIRED - Meningococcal infection; HSP; SLE; scurvy

Question 34

What are the causes of increased platelet destruction?

Answer 34

IMMUNE - ITP; SLE; alloimmune neonatal thrombocytopenia | NON-IMMUNE - haemolytic uraemic syndrome; TTP; CID; congenital heart disease; giant haemangiomas; hypersplenism

Question 35

What are the causes of impaired platelet production?

Answer 35

CONGENITAL - Fanconi anaemia; Wiskott-Aldrich syndrome; Bernard-Soulier syndrome ACQUIRED - Aplastic anaemia; marrow infiltration; drug-induced

Question 36

What is the commonest cause of thrombocytopenia in children?

Answer 36

ITP

Question 37

What are the atypical features one should be aware of when considering a Dx of ITP, as they may point to aplastic anaemia or acute leukaemia?

Answer 37

1. Anaemia 2. Neutropenia 3. Hepatosplenomegaly 4. Marked lymphadenopathy