What is primary autoimmune neutropenia of childhood?

By William Aird

One of the most frequent isolated, chronic, acquired neutropenias diagnosed in infants or toddlers:

  • Diagnosed during the first year of life (median age 8 months, range 3–36 months).
  • Neutrophils can reach levels <0.2 x 109 /L, often accompanied by mild monocytosis.
  • Neutropenia is typically detected during the occurrence of an acute febrile illness.
  • Typically asymptomatic, but some cases neutropenia may be accompanied by minor infections.
  • Serum anti-neutrophil antibodies may be positive.
  • Rarely associated with other autoimmune disease.
  • Usually resolves spontaneously before the age of 5 years, mean duration 17 months.

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