Autosomal dominant severe congenital neutropenia

What is Autosomal dominant severe congenital neutropenia?

A rare primary immunodeficiency disorder characterized by autosomal dominant inheritance, absolute neutrophil counts below 0.5x10E9/L in the peripheral blood (on three separate occasions over a six month period), granulopoiesis maturation arrest at the promyelocyte/myelocyte stage and early-onset, severe, recurrent bacterial infections.

Inheritance type

Autosomal dominant

Age of Onset

  • Infancy
  • Neonatal
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