D2I2.
genetic

Severe congenital neutropenia

Severe neutropenia (SCN), also often known as Kostmann or Kostmann disease, is a group of rare disorders that affect myelopoiesis, causing a congenital form of neutropenia, usually without other physical malformations. SCN manifests in infancy with life-threatening . It causes severe pyogenic infections. It can be caused by inheritance of the ELANE gene, autosomal inheritance of the HAX1 gene. There is an increased risk of and myelodysplastic cancers.

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Causes — why it happens

  • · in genes, depending on type

How it's found

  • ·Blood test, testing

Treatment

  • ·G-CSF, HSCT
Plain-language summary adapted from Wikipedia. Not medical advice.