Congenital agranulocytosis

This code represents a rare, inherited disorder characterized by a severe deficiency or complete absence of neutrophils, a type of white blood cell crucial for fighting infection, present from birth. Patients with this condition experience recurrent, life-threatening bacterial infections due to their compromised immune system.

This code is used for patients diagnosed with congenital agranulocytosis, also known as Kostmann syndrome or severe congenital neutropenia. Documentation should clearly indicate the congenital nature of the neutropenia and the severe deficiency of neutrophils.

• Ensure documentation specifies "congenital" to differentiate from acquired forms of agranulocytosis.
• Do not confuse with other types of neutropenia that are not congenital or as severe.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, especially for management of related infections or treatment.