4th Edition

Chapter 28: The listless infant

An 11-month-old baby boy was brought to hospital because he had been irritable for a week and would not stop crying. For the last 4 days he had a cough, coryza and a fever and now he was short of breath with a swollen abdomen.

The pregnancy and his birth were medically uneventful and both his parents were well but incredibly anxious. His immunizations were up to date and he was gaining weight well.

He was sick, pale, febrile and tachypneic with signs of severe dehydration.

FBC showed:

  • Haemoglobin 53 g/L
  • White cell count 27,000/mm3
  • Platelet count 23,000/mm3

  • 1. What does the peripheral blood film show?

    Correct answer:

    Normocytic normochromic anaemia with a marked leucocytosis comprised of lymphoid blasts.

FISH analysis uses red and green fluorescent probes either side of the breakpoint region of MLL gene on chromosome 11q23. The splitting of the signal confirms a translocation.

Reciprocal chromosomal translocations involving the MLL gene on chromosome 11q23 with a variety of different partner genes on many different chromosomes with over 40 partner genes identified and cloned. The consequences of the chromosomal translocations is usually the formation of hybrid fusion proteins. Reciprocal translocations of 11q23 are a feature of infantile ALL as well as secondary, chemotherapy-induced leukaemias.

He was treated with combination chemotherapy and follow-up examination of his bone marrow at the end of induction therapy showed no morphologic evidence of residual leukaemia and both FISH and chromosome analyses were normal. Consolidation and maintenance chemotherapy followed and he remains in remission 3 years later. Acute lymphocytic leukaemia is the most common childhood leukaemia. Overall, the prospects for cure are very good, with a chance in excess of 80% of a sustained remission.

The illness placed a huge strain on his parent‘s marriage and they are now separated but always turn up together for his follow-up appointments. The breakdown of marriage is a relatively common occurrence in cancer survivors and the parents of children with cancer.

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