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Archives of Disease in Childhood 2006;91:852-858; doi:10.1136/adc.2006.094706
Copyright © 2006 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health.

REVIEW

The investigation and management of chronic neutropenia in children

R M James1, S E Kinsey2

1 Epidemiology and Genetics Unit, Department of Health Sciences, University of York, UK
2 Department of Paediatric Haematology and Oncology, St James’ University Hospital, Leeds, UK

Correspondence to:
Correspondence to:
Dr S E Kinsey
Consultant Paediatric Haematologist, Department of Paediatric Haematology and Oncology, Children’s Day Hospital, St James’ University Hospital, Beckett Street, Leeds LS9 7TF, UK; Sally.Kinsey{at}leedsth.nhs.uk

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.

Abbreviations: 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

Keywords: autoimmune neutropenia; severe chronic neutropenia; cyclical neutropenia; granulocyte colony stimulating factor


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