2026 ICD-10-CM Diagnosis Code D64.4Congenital dyserythropoietic anemia

ICD-10-CM Codes›D50–D89›D60-D64›D64

ICD-10-CM D64.4
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D64.4 is a billable ICD-10-CM diagnosis code for congenital dyserythropoietic anemia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 811 through 812. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Aplastic anemia.

Code Identity

ICD-10-CM Code
D64.4
Billable Status
Yes — Valid for Submission
Code Describes
Congenital dyserythropoietic anemia
Short Description
Congenital dyserythropoietic anemia
Same as the full description in the CMS dataset.
Parent Code
D64
Other anemias

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD60-D64Aplastic and other anemias and other bone marrow failure syndromes
CategoryD64Other anemias
This CodeD64.4Congenital dyserythropoietic anemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • CIMDAG syndrome
  • Congenital dyserythropoietic anemia
  • Congenital dyserythropoietic anemia type IV
  • Congenital dyserythropoietic anemia, type I
  • Congenital dyserythropoietic anemia, type II
  • Congenital dyserythropoietic anemia, type III
  • Cytochrome-c oxidase deficiency
  • Exocrine pancreatic insufficiency
  • GATA binding protein 1 related thrombocytopenia with dyserythropoiesis
  • Giant platelet syndrome
  • Pancreatic insufficiency
  • Pancreatic insufficiency, dyserythropoietic anemia, calvarial hyperostosis syndrome
  • X-linked congenital dyserythropoietic anemia with thrombocytopenia
  • X-linked dyserythropoietic anemia with abnormal platelets and neutropenia

Tabular List NotesGuidance

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

Inclusion Terms

  • Dyshematopoietic anemia (congenital)

Type 1 Excludes

  • Blackfan-Diamond syndrome D61.01
  • Di Guglielmo's disease C94.0

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.

    • Anemia(essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound)
      • congenital
        • dyserythropoietic, dyshematopoietic
    • Anemia(essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound)
      • dyserythropoietic (congenital)
    • Anemia(essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound)
      • dyshematopoietic (congenital)

Clinical ClassificationClinical

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

CCSR BLD003
Aplastic anemia
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Exocrine Pancreatic Insufficiency

    a malabsorption condition resulting from greater than 10% reduction in the secretion of pancreatic digestive enzymes (lipase; proteases; and amylase) by the exocrine pancreas into the duodenum. this condition is often associated with cystic fibrosis and with chronic pancreatitis.

  • 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

Anemia

If you have anemia, your blood does not carry enough oxygen to the rest of your body. The most common cause of anemia is not having enough iron. Your body needs iron to make hemoglobin. Hemoglobin is an iron-rich protein that gives the red color to blood. It carries oxygen from the lungs to the rest of the body.

Read the full article at MedlinePlus

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

Convert D64.4 to ICD-9-CMHistory

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

ICD-9-CM
285.8 Anemia NEC
Approximate The match is approximate rather than exact.

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 D64.4Overview

Is D64.4 (Other anemias) a billable code?

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

What MS-DRG does D64.4 group to?

When congenital dyserythropoietic anemia is the principal diagnosis on an inpatient stay, it groups to MS-DRG 811, 812, with relative weights from 0.9182 to 1.4043 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D64.4?

Under the General Equivalence Mappings, congenital dyserythropoietic anemia converts to ICD-9-CM 285.8 (anemia NEC). The mapping is approximate, so confirm the match fits the documentation.

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.