D70.0 Congenital agranulocytosis

The ICD-10-CM code for Congenital agranulocytosis is D70.0 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

3: Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

Section

D70-D77: Other disorders of blood and blood-forming organs (D70-D77)

Category D70

8 codes (7 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to D70.0:

• Aleukia › congenital
• Leukopenia › familial
• Leukopenia › cyclic
• Leukopenia › infantile genetic
• Disease, diseased › Kostmann's (infantile genetic agranulocytosis)
• Leukopenia › periodic
• Agranulocytosis (chronic) (cyclical) (genetic) (infantile) (periodic) (pernicious) › congenital
• Leukopenia › congenital

Inclusion Terms

• Congenital neutropenia
• Infantile genetic agranulocytosis
• Kostmann's disease

U.S. Hospital Utilization

• An estimated 995 U.S. inpatient stays in 2023 included D70.0 among the documented diagnoses.
• 125 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Chapter 3 TOC entry

3. Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) ............................................................................................. 39

Reserved for future guideline expansion

Reserved for future guideline expansion ...................................................................................... 39

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)