Cyclic neutropenia Please rate this page (bottom of page)

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Cyclic neutropenia is a blood disease with periodic fever and an autosomal dominant inheritance and is considered among the periodic fever syndromes / autoinflammatory diseases. Mutations in the elastase gene (ELA2 = ELANE) on chromosome 19p13.3 have been identified as the cause


Incidence 1-2 / million / year. Prevalence 1-9 / 1.

  • Disease onset before two years of age is typical, but an adult / adult form also occurs

Symptoms and diagnosis

  • Cyclic fever every XNUM. day (21-XNUM days)
  • Duration 5-7 days. Wounds in the mouth (stomatitis)
  • Periodontitis, Gingivitis, pharyngitis
  • Bacterial Sepsis and rare infections. Often Clostridium septicum
  • Diagnosis: neutrophils less than 200 / microL at least three to five consecutive days per cycle for at least three regular periods
  • Test for ELANE gene mutation

Blood tests: Low neutrophil granulocytes

Medical Prognosis / Life expectancy

  • The disease can disappear at the age of 30

Incorrect diagnosis (similar conditions / differential diagnoses)


  • Granulocyte colony stimulating factor (G-CSF)
  • infection Treatment


Auto-inflammatory diseases (

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