Haem: Paediatric haematology Pt.2

Question 1

How is splenic sequestration managed?

Answer 1

• Blood transfusion
• Splenectomy

Parents should be taught how to palpate the spleen and to seek help when the child is acutely unwell with a large spleen

Question 2

What does hyposplenism increase the risk of? How is this managed?

Answer 2

• Infection by encapuslated bacteria
• Prevented with vaccinations and prophylactic antibiotics

Question 3

Which infectious agents are particularly dangerous in children with SCD?

Answer 3

• Pneumococcus
• Parvovirus B19

Question 4

What can parvovrius B19 cause in SCD?

Answer 4

Aplastic anaemia

Question 5

How can pneumococcal infection be prevented in a patient with sickle cell anaemia?

Answer 5

• Vaccination
• Prophylactic antibiotics

Question 6

Why do children with sickle cell anaemia have increased folate demands?

Answer 6

Reduced red cell lifespan

Question 7

What are the principles of managing sickle cell anaemia in children?

Answer 7

• Monitoring with swift recognition and treatment of complications
• Educate parents
• Vaccinations
• Antibiotic prophylaxis
• Folic acid

Question 8

Describe the difference in severity of beta thalassaemia trait and beta thalassaemia major.

Answer 8

• Trait - harmless but genetically important (can impact offspring)
• Major - severe anaemia that is tranfusion-dependent

NOTE: there is an moderate form of the disease called beta-thalassaemia intermedia

Question 9

List some clinical features of beta thalassaemia major.

Answer 9

• Anaemia → heart failure, growth retardation
• Erythropoietic drive → bone expansion, hepatomegaly, splenomegaly
• Iron overload → heart failure, gonadal failure

Question 10

What are the principles of treatment of beta thalassaemia major?

Answer 10

• Accurate diagnosis and family counselling
• Blood transfusion
• Iron chelation therapy
• Consider child as individual an part of family

Question 11

Name an iron-chelating drug

Answer 11

Desferrioxamine

Question 12

List some types of inherited haemolytic anaemia.

Answer 12

• Red cell membrane - hereditary spherocytosis, hereditary eliptocytosis
• Haemoglobin molecule - sickle cell anaemia, thalassaemia
• Glycolytic pathway - pyruvate kinase deficiency
• Pentose shunt - G6PD deficiency

Question 13

Give an example of acquired congential haemolytic anaemia

Answer 13

Haemolytic disease of the newborn

Question 14

What should you look for when investigating a patient with suspected haemolytic anaemia?

Answer 14

• Is there anaemia?
• Is there evidence of increased red cell turnover? (e.g. jaundice, splenomegaly)
• Is there evidence of increased red cell production? (e.g. increased reticulocyte count, bone expansion)
• Are there abnormal cells?

Question 15

What type of anaemia would also cause a low reticulocyte count?

Answer 15

Aplastic

Question 16

Aside from the haemolysis, what else contributes to anaemia in sickle cell anaemia?

Answer 16

• HbS has a low affinity for oxygen meaning that is releases oxygen readily to tissues
• This reduces EPO synthesis

Question 17

List some triggers for haemolysis in G6PD deficiency.

Answer 17

• Infections
• Drugs
• Naphthalene
• Fava beans

Question 18

What is the inheritance pattern of G6PD deficiency?

Answer 18

X-linked recessive

Question 19

What 2 signs would be seen on the blood film of someone with G6PD deficiency?

Answer 19

Question 20

What are the two important types of acquired haemolytic anaemia in children?

Answer 20

• Autoimmune haemolytic anaemia
• Haemolytic uraemic syndrome