2026 ICD-10-CM Diagnosis Code D56.4Hereditary persistence of fetal hemoglobin [HPFH]
ICD-10-CM Codes›D50–D89›D55-D59›D56
- Billable — Valid for Submission
- Chronic Condition
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.
D56.4 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 48 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 48, ESRD (V21) category 48, and ESRD (V24) category 48 for payment year 2026.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
D56.4 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2026 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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.
Hemoglobin See Also: condition;
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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.
Code HistoryHistory
Questions About D56.4Overview
What is the ICD-10 code for hereditary persistence of fetal hemoglobin [HPFH]?
The ICD-10-CM code for hereditary persistence of fetal hemoglobin [HPFH] is D56.4 (sometimes written as D564). It is billable on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
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.
Does D56.4 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. D56.4 mapped to HCC 48 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 48 (Coagulation Defects and Other Specified Hematological Disorders), ESRD (V21) category 48 (Coagulation Defects and Other Specified Hematological Disorders), and ESRD (V24) category 48 (Coagulation Defects and Other Specified Hematological Disorders).
