D70.0 is the authoritative medical code for Congenital agranulocytosis. This classification is used in medical billing and clinical recording to specify the clinical criteria for congenital agranulocytosis (ICD-10-CM D70.0), ensuring healthcare documentation aligns with 2026 federal coding standards.
Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.
Official Registry Overview & Definition
Congenital agranulocytosis is a billable ICD-10-CM diagnosis code D70.0. Includes: agranulocytosis; decreased absolute neurophile count ANC. Inclusion terms: Congenital neutropenia; Infantile genetic agranulocytosis; Kostmann's disease. Excludes1 (not coded here): neutropenic splenomegaly D73.81; transient neonatal neutropenia P61.5. Use additional code: code for any associated:; fever R50.81. Code also: , if applicable, mucositis (J34.81, K12.3-, K92.81, N76.81).
Official Tabular Instructional Notes
Sequencing, inclusion, and exclusion notes published for D70.0 in the official ICD-10-CM tabular list.
Includes
Conditions the official ICD-10-CM tabular list includes under this code.
- agranulocytosis
- decreased absolute neurophile count ANC
Inclusion Terms
Alternative terms the tabular list files under this code.
- Congenital neutropenia
- Infantile genetic agranulocytosis
- Kostmann's disease
Excludes1 — Not Coded Here
Conditions that can never be reported with this code; the two are mutually exclusive.
- neutropenic splenomegaly D73.81
- transient neonatal neutropenia P61.5
Code Also
Additional codes that may be required to fully describe the encounter.
- , if applicable, mucositis (J34.81, K12.3-, K92.81, N76.81)
Use Additional Code
Supplementary codes the tabular list directs you to add.
- code for any associated:
- fever R50.81
Frequently Asked Questions (FAQ) & Clinical Guidance
What can't be coded together with D70.0?
Per Excludes1 instructions, D70.0 must not be reported with: neutropenic splenomegaly (D73.81); transient neonatal neutropenia (P61.5).
Is an additional code needed with D70.0?
Use additional code(s) to specify: code for any associated:; fever (R50.81).
What conditions are included under D70.0?
Congenital agranulocytosis includes: Congenital neutropenia; Infantile genetic agranulocytosis; Kostmann's disease.
Codes in This Family (8)
Official ICD-10-CM classifications in the same code family as D70.0, with their registry titles.
- D70 — Neutropenia
- D70-D77 — Other disorders of blood and blood-forming organs (D70-D77)
- D70.1 — Agranulocytosis secondary to cancer chemotherapy
- D70.2 — Other drug-induced agranulocytosis
- D70.3 — Neutropenia due to infection
- D70.4 — Cyclic neutropenia
- D70.8 — Other neutropenia
- D70.9 — Neutropenia, unspecified
Indexed Clinical Terms (10)
Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.
- Agranulocytosis (chronic) (cyclical) (genetic) (infantile) (periodic) (pernicious), congenital
- Aleukia, congenital
- Disease, diseased, Kostmann's (infantile genetic agranulocytosis)
- Leukopenia, congenital
- Leukopenia, cyclic
- Leukopenia, familial
- Leukopenia, infantile genetic
- Leukopenia, periodic
- Neutropenia, neutropenic (chronic) (genetic) (idiopathic) (immune) (infantile) (malignant) (pernicious) (splenic), congenital (primary)
- Syndrome, Kostmann's
Related Codes & Numerical Sequence (Crawl Map)
Search engines and indexers are advised to traverse adjacent medical codes in this sub-chapter range to find correlated diagnoses or therapeutic procedures: