A 4-year-old girl with sickle cell anaemia presents with abdominal pain

A 4-year-old girl with sickle cell anaemia presents with abdominal pain. On examination, she is noted to have splenomegaly and is clinically anaemic. What is the most likely diagnosis?

Liver cirrhosis

Parvovirus infection

Sequestration crisis

Salmonella infection

Thrombotic crisis

During a sequestration crisis, the sickle cells cause the spleen to become grossly enlarged causing the abdominal pain as present in this case. This is more common in early childhood as repeated sequestration and infarction of the spleen during childhood gradually results in an auto-splenectomy. A sequestration crisis may result in severe anaemia, marked pallor and cardiovascular collapse due to loss of effective circulating volume.

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Sickle-cell crises

Sickle cell anaemia is characterised by periods of good health with intervening crises

A number of types of crises are recognised:

  • thrombotic, ‘painful crises’
  • sequestration
  • acute chest syndrome
  • aplastic
  • haemolytic

Thrombotic crises

  • also known as painful crises or vaso-occlusive crises
  • precipitated by infection, dehydration, deoxygenation
  • infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain

Sequestration crises

  • sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia

Acute chest syndrome

  • dyspnoea, chest pain, pulmonary infiltrates, low pO2
  • the most common cause of death after childhood

Aplastic crises

  • caused by infection with parvovirus
  • sudden fall in haemoglobin

Haemolytic crises

  • rare
  • fall in haemoglobin due an increased rate of haemolysis