Paediatric Haematology Flashcards
What are causes of polycythaemia in fetuses and neonates?
Twin-to-twin transfusion
Intrauterine hypoxia
Placental insufficiency
What are unique causes of anaemia in fetuses and neonates?
Twin-to-twin transfusion
Fetal-to-maternal transfusion
Parvovirus infection (virus not cleared by immature immune system)
Haemorrhage from the cord or placenta
What is the type of congenital leukaemia associated with Down’s Syndrome?
This specific type of neonatal leukaemia (also sometimes called transient abnormal myelopoiesis or TAM) differs greatly from leukaemia in older infants or children.
Newborn babies, in contrast to adults, have:
A. A higher Hb
B. A lower WBC
C. Smaller red blood cells
D. The same percentage of haemoglobin F
A. A higher Hb
Which globin chains are haemoglobin A made of and when is it present?
α2β2
Late fetus, infant, child and adult
Which globin chains are haemoglobin A2 made of and when is it prevelant?
α2δ2
Infant, child and adult
Which globin chains are haemoglobin F made of and when is it prevalent?
α2γ2
Fetus and infant
Why does sickle cell anaemia not manifest at birth?
Clinical features become manifest as gamma chain production and haemoglobin F synthesis decrease and betaS and haemoglobin S production increase.
Why does sickle cell anaemia present differently in infants and children compared to adults?
The infant still has a functioning spleen—splenic sequestration can occur.
What is splenic sequestration?
Splenic sequestration is the acute pooling of a large percentage of circulating red cells in the spleen.
The spleen enlarges acutely.
The Hb falls acutely and death can occur.
This doesn’t happen in older children and adults because recurrent infarction has left the spleen small and fibrotic.
Why does folic acid matter more in a child with sickle cell disease than in a normal child or an adult?
Hyperplastic erythropoiesis requires folic acid.
Growth spurts require folic acid.
Red cell life span is shorter so anaemia can rapidly worsen.
Complications of sickle cell anaemia that are more common in adults than children include:
A. Hand-foot syndrome
B. Hyposplenism
C. Red cell aplasia
D. Splenic sequestration
E. Stroke
B. Hyposplenism
Siblings with sickle cell anaemia present simultaneously with severe anaemia and a low reticulocyte count—likely diagnosis?
A. Splenic sequestration
B. Parvovirus B19 infection
C. Folic acid deficiency
D. Haemolytic crisis
E. Vitamin B12 deficiency
B. Parvovirus B19 infection
A 6-year-old Afro-Caribbean boy presents with chest and abdominal pain; Hb is 63 g/l, MCV 85 fl and blood film shows sickle cells—likely diagnosis?
A. Sickle cell trait
B. Sickle cell anaemia
C. Sickle cell/beta thalassaemia
B. Sickle cell anaemia
What can inherited haemolytic anaemias be due to?
Defects in:
- Red cell membrane
- Haemoglobin molecule
- Red cell enzymes—glycolytic pathway
- Red cell enzymes—pentose shunt
- Not to mention other rare conditions that you really don’t want to hear about
