D70.0 Congenital agranulocytosis

Billable ICD-10-CM code, FY2026.

Applicable to

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

Applies from D70 Neutropenia

Includes

• agranulocytosis
• decreased absolute neurophile count (ANC)

Excludes1 (never code together)

• 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)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 115 — Specified Immunodeficiencies and White Blood Cell Disorders
• CMS-HCC V22: HCC 47 — Disorders of Immunity
• ESRD V24: HCC 47

MS-DRG index

Defines the logic of DRG(s) 808-810 in MDC 16.

Approximate ICD-9-CM (GEMs)

• 288.01 Congenital neutropenia

Code annotations containing back-references to D70.0

Back-references to D70.0

• Type 1 Excludes: P61.5 Transient neonatal neutropenia ("congenital neutropenia (nontransient) (D70.0)")

Back-references to D70

• Type 2 Excludes: D61 Other aplastic anemias and other bone marrow failure syndromes ("neutropenia (D70.-)")
• Type 1 Excludes: D72 Other disorders of white blood cells ("neutropenia (D70)")
• Type 1 Excludes: D72.81 Decreased white blood cell count ("neutropenia (D70.-)")
• Code First: R50.81 Fever presenting with conditions classified elsewhere ("neutropenia (D70.-)")

Diagnosis Index entries for D70.0

• 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

Nearby codes

• D70 Neutropenia
• D70.0 Congenital agranulocytosis (this code)
• 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