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D56.5 ICD-10-CM Code: Hemoglobin E-beta thalassemia

D56.5 maps to CMS-HCC V28 108. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · HCC coding software

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Code lookupD56.5

FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Hemolytic anemias (D55-D59)

D56.5

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Hemoglobin E-beta thalassemia

A blood disorder where a person inherits hemoglobin E from one parent and a thalassemia gene from the other, causing mild to moderate anemia and abnormal red blood cells.

Buddy the Bee presenting code insight

Buddy Insight

Hemoglobin E-beta thalassemia is a compound heterozygous condition where one parent contributes a hemoglobin E gene and the other a beta thalassemia gene.

CMS-HCC V28

HCC 108

Coefficient HCC 108: 0.146 (Community Non-Dual Aged (CNA))

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

HCC 071

Code-level coefficient reference

ESRD/PACE

HCC 48

Code-level coefficient reference

RXHCC

N/A

Not mapped

Inclusion Terms

Official

No inclusion terms are included in this display for D56.5. Check the code and parent instructions in the Code Book.

Excludes 2

Official
  • autoimmune disease (systemic) NOS (M35.9)Inherited from D50-D89
  • certain conditions originating in the perinatal period (P00-P96)Inherited from D50-D89
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)Inherited from D50-D89
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)Inherited from D50-D89
  • endocrine, nutritional and metabolic diseases (E00-E88)Inherited from D50-D89
  • human immunodeficiency virus [HIV] disease (B20)Inherited from D50-D89
  • injury, poisoning and certain other consequences of external causes (S00-T88)Inherited from D50-D89
  • neoplasms (C00-D49)Inherited from D50-D89
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)Inherited from D50-D89

Includes

Official

No Includes notes are included in this display for D56.5. Check the code and parent instructions in the Code Book.

Excludes 1

Official
  • beta thalassemia (D56.1)
  • beta thalassemia minor (D56.3)
  • beta thalassemia trait (D56.3)
  • delta-beta thalassemia (D56.2)
  • delta-beta thalassemia trait (D56.3)
  • hemoglobin E disease (D58.2)
  • other hemoglobinopathies (D58.2)
  • sickle-cell beta thalassemia (D57.4-)
  • sickle-cell thalassemia (D57.4-)Inherited from D56

Code First

Official

No Code First sequencing instructions are included in this display for D56.5. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for D56.5. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for D56.5. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Document the compound heterozygous state with hemoglobin electrophoresis showing hemoglobin E and elevated fetal hemoglobin, supported by genetic testing when available.
Specify clinical severity (mild, moderate, or severe/transfusion-dependent), hemoglobin levels, mean corpuscular volume, transfusion schedule, iron chelation therapy, and ferritin monitoring results.
Record complications including splenomegaly, iron overload, and endocrinopathies.

MEAT Support

HCC Buddy guidance
Document the compound heterozygous state with hemoglobin electrophoresis showing hemoglobin E and elevated fetal hemoglobin, supported by genetic testing when available.
Specify clinical severity (mild, moderate, or severe/transfusion-dependent), hemoglobin levels, mean corpuscular volume, transfusion schedule, iron chelation therapy, and ferritin monitoring results.
Record complications including splenomegaly, iron overload, and endocrinopathies.

Audit Caution

HCC Buddy guidance
Clinical severity varies dramatically based on the specific beta thalassemia mutation, so documentation of clinical phenotype is essential. Do not code hemoglobin E trait alone as D56.5 -
this code requires both the hemoglobin E variant and a co-inherited beta thalassemia gene. Ensure the provider documents both genetic components.

Common Mistakes

HCC Buddy guidance
D56.5 vs. D56.1 (Beta thalassemia) -
D56.5 is specifically for the combination of hemoglobin E plus beta thalassemia, not beta thalassemia alone. D56.5 vs. D58.2 (Other hemoglobinopathies) -
hemoglobin E disease without thalassemia co-inheritance is coded separately. D56.5 vs. D57.40 (Sickle-cell thalassemia) -
different hemoglobin variant combined with thalassemia.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is D56.5 an HCC code?

Yes. D56.5 (Hemoglobin E-beta thalassemia) maps to HCC 108, Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 0.146. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.

Coder answer: D56.5 is billable and maps to V28 HCC 108, Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.

Code
D56.5
Description
Hemoglobin E-beta thalassemia
HCC (V28)
HCC 108 — Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major
RAF reference coefficient
0.146
Billable
Yes
Payment year
2026

HCC Category Mapping

V28HCC 108, Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major
0.146
ESRDHCC 48, Coagulation Defects and Other Specified Hematological Disorders
Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work D56.5 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for D56.5

For D56.5, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Coder workflow notes

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What This Code Means

D56.5 is the ICD-10-CM diagnosis code for hemoglobin e-beta thalassemia. A blood disorder where a person inherits hemoglobin E from one parent and a thalassemia gene from the other, causing mild to moderate anemia and abnormal red blood cells. D56.5 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering hemolytic anemias (d55-d59).

Under the CMS-HCC V28 risk adjustment model, D56.5 maps to Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major (HCC 108) with a source-labeled community, non-dual, aged reference coefficient of 0.146. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.

Document whether the patient has hemoglobin E trait or disease to ensure accurate code selection. For D56.5, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for D56.5 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Document whether the patient has hemoglobin E trait or disease to ensure accurate code selection
  • Verify the specific type of beta thalassemia component (thalassemia major, minor, or intermedia) when documented

Clinical Significance

Hemoglobin E-beta thalassemia is a compound heterozygous condition where one parent contributes a hemoglobin E gene and the other a beta thalassemia gene. It is the most common form of severe thalassemia worldwide, particularly prevalent in Southeast Asian populations. Clinical severity varies from asymptomatic to transfusion-dependent, depending on the beta thalassemia mutation type (beta-zero vs. beta-plus), making accurate characterization essential for management planning.

Documentation Requirements

  • Document the compound heterozygous state with hemoglobin electrophoresis showing hemoglobin E and elevated fetal hemoglobin, supported by genetic testing when available.
  • Specify clinical severity (mild, moderate, or severe/transfusion-dependent), hemoglobin levels, mean corpuscular volume, transfusion schedule, iron chelation therapy, and ferritin monitoring results.
  • Record complications including splenomegaly, iron overload, and endocrinopathies.

Excludes 1, Do NOT code together

  • beta thalassemia (D56.1)
  • beta thalassemia minor (D56.3)
  • beta thalassemia trait (D56.3)
  • delta-beta thalassemia (D56.2)
  • delta-beta thalassemia trait (D56.3)
  • hemoglobin E disease (D58.2)
  • other hemoglobinopathies (D58.2)
  • sickle-cell beta thalassemia (D57.4-)

Commonly Confused Codes

  • D56.5 vs. D56.1 (Beta thalassemia) -
  • D56.5 is specifically for the combination of hemoglobin E plus beta thalassemia, not beta thalassemia alone. D56.5 vs. D58.2 (Other hemoglobinopathies) -
  • hemoglobin E disease without thalassemia co-inheritance is coded separately. D56.5 vs. D57.40 (Sickle-cell thalassemia) -
  • different hemoglobin variant combined with thalassemia.

Child Codes

Code Hierarchy

D56ThalassemiaD56.5Hemoglobin E-beta thalassemia
D56.5Hemoglobin E-beta thalassemia

Also searched as

  • D56 5
  • D565

For D56.5, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

D56.5 maps to CMS-HCC V28 category 108, Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major. See the ICD-10 to HCC mapping hub for how the V28 crosswalk works. The mapping identifies a payment HCC category for D56.5. Review its source-labeled HCC coefficient above, check the RAF Calculator with complete member context for CMS-HCC V28 PY2026, and confirm the documentation the chart needs before the code is submitted. HCC Buddy shows no RAF score unless every required source and calculation check passes.

Work D56.5 in HCC Buddy

Open D56.5 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 7 days to try everything, no card required.