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Concept information

disease > genetic disease > hereditary disease > Kostmann syndrome
disease > immunopathology > immune deficiency > Kostmann syndrome
disease > congenital disease > congenital neutropenia > Kostmann syndrome
disease > hemopathy > congenital neutropenia > Kostmann syndrome
... > disease > hemopathy > leukopenia > neutropenia > congenital neutropenia > Kostmann syndrome

Preferred term

Kostmann syndrome  

Definition

  • Severe congenital neutropenia (SCN), also often known as Kostmann syndrome or disease, is a group of rare disorders that affect myelopoiesis, causing a congenital form of neutropenia, usually without other physical malformations. (Wikipedia)

Broader concept

Synonym(s)

  • Kostmann disease

In other languages

  • syndrome de Kostmann

    French

  • agranulocytose héréditaire de Kostmann
  • agranulocytose infantile de Kostmann
  • maladie de Kostmann
  • neutropénie congénitale sévère

URI

http://data.loterre.fr/ark:/67375/VH8-CQBS1P5B-7

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