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Adult idiopathic neutropenia

Disease definition

A rare acquired immunodeficiency disease characterized by adult-onset absolute neutrophil counts less than 1.5 x 10^9/L on at least 3 occasions in a 3 month period that cannot be attributable to drugs or a specific genetic, infectious, inflammatory, autoimmune or malignant cause. Recurrent aphtous stomatitis and a history of mild bacterial infections are typically associated. A benign outcome with a low rate of severe infections and no secondary malignancies is observed.

ORPHA:2688

Classification level: Disorder
  • Synonym(s):
    • Adult chronic idiopathic neutropenia
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: D70
  • OMIM: 607847
  • UMLS: C1842930
  • MeSH: -
  • GARD: -
  • MedDRA: -
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