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The investigation and management of chronic neutropenia in children

Abstract

Unravelling the cause of a neutropenia poses a complex diagnostic challenge. The differential diagnosis ranges from life threatening disease to transient benign causes of little clinical significance. This review offers a practical guide to investigating the neutropenic child, and highlights features that merit specialist referral. Therapeutic options, the role of long term follow up, and the complications of severe chronic neutropenia are considered.

  • ANC, absolute neutrophil count
  • FBC, full blood count
  • G-CSF, granulocyte colony stimulating factor
  • GSD1b, glycogen storage disease 1b
  • HSCT, haemopoietic stem cell transplant
  • MDS/AML, myelodysplasia or acute myeloid leukaemia
  • SCN, severe chronic neutropenia
  • SCNIR, severe chronic neutropenia international registry
  • autoimmune neutropenia
  • severe chronic neutropenia
  • cyclical neutropenia
  • granulocyte colony stimulating factor

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    BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health