Cyclic neutropenia

This code represents a rare, congenital disorder characterized by recurrent, periodic episodes of severe neutropenia, typically occurring every 21 days and lasting 3-6 days. During these cycles, the bone marrow temporarily fails to produce adequate neutrophils, leading to increased susceptibility to infection. Patients often present with fever, oral ulcers, pharyngitis, and skin infections during neutropenic phases.

Use this code for patients diagnosed with cyclic neutropenia based on clinical presentation and laboratory findings demonstrating cyclical fluctuations in neutrophil counts. Documentation should clearly indicate the periodic nature of the neutropenia, often supported by serial complete blood counts (CBCs) over several weeks or months. This code is appropriate for both initial diagnosis and ongoing management of the condition.

• Ensure documentation specifies "cyclic" neutropenia to differentiate from other forms of neutropenia.
• Do not confuse with acquired or drug-induced neutropenia, which have distinct codes.
• Code any associated infections or complications separately, linking them to the neutropenia if clinically appropriate.