2026 ICD-10-CM Diagnosis Code D56.4Hereditary persistence of fetal hemoglobin [HPFH]

ICD-10-CM Codes›D50–D89›D55-D59›D56

ICD-10-CM D56.4
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D56.4 is a billable ICD-10-CM diagnosis code for hereditary persistence of fetal hemoglobin [HPFH]. 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 Hemolytic anemia.

Code Identity

ICD-10-CM Code
D56.4
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary persistence of fetal hemoglobin [HPFH]
Short Description
Hereditary persistence of fetal hemoglobin [HPFH]
Same as the full description in the CMS dataset.
Parent Code
D56
Thalassemia

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD55-D59Hemolytic anemias
CategoryD56Thalassemia
This CodeD56.4Hereditary persistence of fetal hemoglobin [HPFH]

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • A>gamma< beta^+^ HPFH AND beta^0^ thalassemia in cis
  • Beta plus thalassemia
  • Beta zero thalassemia
  • Delta beta thalassemia
  • Delta beta zero thalassemia
  • Hereditary persistence of fetal hemoglobin
  • Hereditary persistence of fetal hemoglobin delta beta plus thalassemia
  • Hereditary persistence of fetal hemoglobin G gamma beta plus thalassemia
  • Hereditary persistence of fetal hemoglobin thalassemia
  • Hereditary persistence of fetal hemoglobin with sickle cell disease syndrome
  • Hereditary persistence of fetal hemoglobin, intellectual disability syndrome
  • HPFH A gamma beta^+^ thalassemia
  • HPFH deletion type
  • HPFH linked to beta-globulin gene cluster
  • HPFH nondeletion type
  • HPFH unlinked to beta-globulin gene cluster

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.

    • Hemoglobin
      • fetal, hereditary persistence (HPFH)
    • Persistence, persistent(congenital)
      • fetal
        • hemoglobin, hereditary (HPFH)
    • Persistence, persistent(congenital)
      • hemoglobin, fetal (hereditary) (HPFH)

Clinical ClassificationClinical

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

CCSR BLD002
Hemolytic anemia
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Hereditary Persistence of Fetal Hemoglobin

    the persistence of substantial fetal hemoglobin production into adulthood, usually associated with hemoglobinopathies due to mutations in the alpha and/or beta chain of hemoglobin.

Patient EducationClinical

Thalassemia

Thalassemias are inherited blood disorders. If you have one, your body makes fewer healthy red blood cells and less hemoglobin. Hemoglobin is a protein that carries oxygen to the body. That leads to anemia. Thalassemias occur most often among people of Italian, Greek, Middle Eastern, Southern Asian, and African descent.

Read the full article at MedlinePlus

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

Convert D56.4 to ICD-9-CMHistory

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

ICD-9-CM
282.7 Hemoglobinopathies 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 D56.4Overview

Is D56.4 (Thalassemia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary persistence of fetal hemoglobin [HPFH] on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does D56.4 group to?

When hereditary persistence of fetal hemoglobin [HPFH] 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 D56.4?

Under the General Equivalence Mappings, hereditary persistence of fetal hemoglobin [HPFH] converts to ICD-9-CM 282.7 (hemoglobinopathies 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.