The Child with Pallor

Question 1

What are some foods that are high in iron? What foods help absorption? What inhibits it?

Answer 1

• High iron foods - red meats, oily fish, liver, kidney
• Moderate iron foods - legumes, leafy greens, dried fruit and nuts
• Absorption increased when eaten with fresh fruit and vegetables, inhibited by tannins (tea, wine) and milk

Question 2

What is the inheritance of B-thalassaemia? What does the gene defect cause?

Answer 2

• Autosomal dominant
• B⁰B⁰ - HbA2 (a4) is overproduced and forms insoluble aggregates in RBC membranes
  
  • Ineffective erythropoiesis and premature clearance by the spleen

Question 3

What kind of FBE abnormality does haemochromatosis cause? What is the diagnostic test?

Answer 3

• FBE - hypochromic microcytosis without significant anaemia
• Hb electrophoresis or HPLC

Question 4

How is B-thalassaemia major treated?

Answer 4

• Repeated transfusion maintaining Hb above 10g/dL
• Iron chelation therapy (desferrioxamine)

Question 5

What are some of the clinical features of leukaemia?

Answer 5

• Bone marrow crowding features
  
  • Space-occupying: bone pain, limp, refusal to weight bear
  • Decreased RBCs: fatigue, pallor
  • Decreased platelets: easy bruising, bleeding, petechiae
  • Decreased WBCs: fever, infection
  • CNS involvement: headache, nausea, vomiting, irritability
• Examination
  
  • Lymphadenopathy, hepatosplenomegaly
  • Testicular involvement (painless, unilateral)