2026 ICD-10-CM Diagnosis Code D70.0Congenital agranulocytosis

ICD-10-CM Codes›D50–D89›D70-D77›D70

ICD-10-CM D70.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D70.0 is a billable ICD-10-CM diagnosis code for congenital agranulocytosis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 808 through 810. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Diseases of white blood cells.

Code Identity

ICD-10-CM Code
D70.0
Billable Status
Yes — Valid for Submission
Code Describes
Congenital agranulocytosis
Short Description
Congenital agranulocytosis
Same as the full description in the CMS dataset.
Parent Code
D70
Neutropenia

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD70-D77Other disorders of blood and blood-forming organs
CategoryD70Neutropenia
This CodeD70.0Congenital agranulocytosis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Autosomal dominant severe congenital neutropenia
  • Autosomal recessive severe congenital neutropenia due to CSF3R deficiency
  • Autosomal recessive severe congenital neutropenia due to CXCR2 deficiency
  • Autosomal recessive severe congenital neutropenia due to G6PC3 deficiency
  • Autosomal recessive severe congenital neutropenia due to JAGN1 deficiency
  • Complete deafness
  • Congenital deafness
  • Congenital dyserythropoietic anemia
  • Congenital neutropenia
  • Congenital neutropenia, myelofibrosis, nephromegaly syndrome
  • Giant platelet syndrome
  • Kostmann syndrome
  • Myelofibrosis
  • Neutropenia, monocytopenia, deafness syndrome
  • Onycho-tricho-dysplasia neutropenia syndrome
  • Periodontitis due to congenital neutropenia
  • Periodontitis due to infantile genetic agranulocytosis
  • Trichothiodystrophy
  • X-linked dyserythropoietic anemia with abnormal platelets and neutropenia
  • X-linked severe congenital neutropenia

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • 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

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD007
Diseases of white blood cells
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • CDAN1 Gene|CDAN1|CDAN1|Congenital Dyserythropoietic Anemia, Type I Gene

    this gene may be involved in nuclear membrane maintenance.

  • CDAN1 wt Allele|CDA-I|CDA1|CDAI|Codanin Gene|Congenital Dyserythropoietic Anemia, Type I wt Allele|DLT|Discs Lost Homolog Gene|Discs Lost, Drosophila, Homolog of Gene|PRO1295|UNQ664/PRO1295

    human cdan1 wild-type allele is located in the vicinity of 15q15.2 and is approximately 14 kb in length. this allele, which encodes codanin-1 protein, may play a role in the maintenance of the nuclear envelope. mutation of the gene is associated with congenital dyserythropoietic anemia type i.

  • Congenital Dyserythropoietic Anemia

    a rare group of disorders that result in anemia that is caused by ineffective erythropoiesis, which is associated with multinuclear erythroblasts, and which may present in childhood. the most common mutations are in the cdan1 and sec23b genes.

  • Congenital Dyserythropoietic Anemia Type II|CDA II|CDAN2|HEMPAS|Hereditary Erythroblastic Multinuclearity with Positive Acidified-Serum Test|SEC23B-CDG

    an autosomal recessive subtype of congenital dyserythropoietic anemia caused by mutation(s) in the sec23b gene, encoding protein transport protein sec23b.

  • Congenital Dyserythropoietic Anemia Type IV|CDAN4

    an autosomal dominant sub-type of congenital dyserythropoietic anemia caused by mutation(s) in the klf1 gene, encoding krueppel-like factor 1.

  • SEC23B wt Allele|CDA-II|CDAII|CDAN2|Congenital Dyserythropoietic Anemia, Type II Gene|HEMPAS|RP11-379J5.1|Sec23 Homolog B (S. cerevisiae) wt Allele

    human sec23b wild-type allele is located in the vicinity of 20p11.23 and is approximately 54 kb in length. this allele, which encodes protein transport protein sec23b, is involved in the transport of vesicles from the endoplasmic reticulum to the golgi. mutation of the gene is associated with congenital dyserythropoietic anemia type ii.

Patient EducationClinical

Blood Disorders

Your blood is living tissue made up of liquid and solids. The liquid part, called plasma, is made of water, salts and protein. Over half of your blood is plasma. The solid part of your blood contains red blood cells, white blood cells and platelets.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D70.0 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
288.01 Congenital neutropenia
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About D70.0Overview

Is D70.0 (Neutropenia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report congenital agranulocytosis on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does D70.0 group to?

When congenital agranulocytosis is the principal diagnosis on an inpatient stay, it groups to MS-DRG 808, 809, 810, with relative weights from 1.0466 to 2.2079 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D70.0?

Under the General Equivalence Mappings, congenital agranulocytosis converts to ICD-9-CM 288.01 (congenital neutropenia). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.