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D56.1 ICD-10-CM Code: Beta thalassemia

D56.1 maps to CMS-HCC V28 108. A source-labeled RAF reference is available. Documentation must support MEAT. MEAT criteria · RAF Calculator · HCC Buddy coding tools

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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)

D56.1

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

Beta thalassemia

A genetic blood disorder where the body cannot make enough beta-globin chains, which are part of hemoglobin. This causes moderate to severe anemia and may require regular blood transfusions.

Buddy the Bee presenting code insight

Buddy Insight

Beta thalassemia results from mutations reducing or eliminating beta-globin chain production, causing ineffective erythropoiesis and hemolytic anemia.

CMS-HCC V28

HCC 108

Code-level coefficient reference

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

Code Book Path

Official
D56Thalassemia
D56.1Beta thalassemia

Inclusion Terms

Official
  • Beta thalassemia major
  • Cooley's anemia
  • Homozygous beta thalassemia
  • Severe beta thalassemia
  • Thalassemia intermedia

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D56.1 in this effective period.

Related Child Codes

Official
D56.0Alpha thalassemia
D56.2Delta-beta thalassemia
D56.3Thalassemia minor
D56.4Hereditary persistence of fetal hemoglobin [HPFH]
D56.5Hemoglobin E-beta thalassemia

Includes

Official

ICD-10-CM does not list Includes notes for D56.1 in this effective period.

Excludes 1

Official
  • beta thalassemia minor (D56.3)
  • beta thalassemia trait (D56.3)
  • delta-beta thalassemia (D56.2)
  • hemoglobin E-beta thalassemia (D56.5)
  • sickle-cell beta thalassemia (D57.4-)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for D56.1 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D56.1 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D56.1 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Documentation must specify the beta thalassemia subtype (major, intermedia, or minor), supported by hemoglobin electrophoresis, genetic testing, or molecular analysis.
Record transfusion schedule and frequency, iron chelation therapy, ferritin levels, cardiac and hepatic iron assessment results, and complications including endocrinopathies, osteoporosis, or extramedullary hematopoiesis.
Annual comprehensive assessment documentation supports recapture.

MEAT Support

HCC Buddy guidance
Documentation must specify the beta thalassemia subtype (major, intermedia, or minor), supported by hemoglobin electrophoresis, genetic testing, or molecular analysis.
Record transfusion schedule and frequency, iron chelation therapy, ferritin levels, cardiac and hepatic iron assessment results, and complications including endocrinopathies, osteoporosis, or extramedullary hematopoiesis.
Annual comprehensive assessment documentation supports recapture.

Audit Caution

HCC Buddy guidance
Do not confuse beta thalassemia major with thalassemia trait/minor, which has markedly different resource utilization. Ensure the subtype is documented -
major and intermedia have very different clinical courses. Iron overload complications should be coded separately for complete clinical picture.

Common Mistakes

HCC Buddy guidance
D56.1 vs. D56.0 (Alpha thalassemia) -
different globin chain affected. D56.1 vs. D56.5 (Hemoglobin E-beta thalassemia) -
D56.5 is for the specific compound heterozygous state with hemoglobin E. D56.1 vs. D57.40 (Sickle-cell thalassemia without crisis) -
D57.40 is for co-inheritance of sickle cell and thalassemia genes. D56.1 vs. D56.3 (Thalassemia minor) -

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 D56.1 an HCC code?

Yes. D56.1 (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: D56.1 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
D56.1
Description
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 D56.1 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT Criteria for D56.1

For D56.1 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • 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

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed D56.1 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

D56.1 is the ICD-10-CM diagnosis code for beta thalassemia. A genetic blood disorder where the body cannot make enough beta-globin chains, which are part of hemoglobin. This causes moderate to severe anemia and may require regular blood transfusions. D56.1 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, D56.1 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. No V24 mapping is shown for D56.1; use the applicable model and payment year when reviewing the V28 mapping. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition. 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.

Specify the type: beta thalassemia major, intermedia, or minor based on clinical severity. Because D56.1 maps to an HCC category, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) before the diagnosis is used for risk adjustment. 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 D56.1 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Specify the type: beta thalassemia major, intermedia, or minor based on clinical severity
  • Document transfusion requirements and any complications like iron overload

Clinical Significance

Beta thalassemia results from mutations reducing or eliminating beta-globin chain production, causing ineffective erythropoiesis and hemolytic anemia. Beta thalassemia major (Cooley anemia) is transfusion-dependent from early childhood, while beta thalassemia intermedia presents with moderate anemia that may or may not require transfusions. The condition carries significant morbidity from iron overload, skeletal changes, and organ damage, making it a high-acuity chronic condition.

Documentation Requirements

  • Documentation must specify the beta thalassemia subtype (major, intermedia, or minor), supported by hemoglobin electrophoresis, genetic testing, or molecular analysis.
  • Record transfusion schedule and frequency, iron chelation therapy, ferritin levels, cardiac and hepatic iron assessment results, and complications including endocrinopathies, osteoporosis, or extramedullary hematopoiesis.
  • Annual comprehensive assessment documentation supports recapture.

Excludes 1, Do NOT code together

  • beta thalassemia minor (D56.3)
  • beta thalassemia trait (D56.3)
  • delta-beta thalassemia (D56.2)
  • hemoglobin E-beta thalassemia (D56.5)
  • sickle-cell beta thalassemia (D57.4-)

Commonly Confused Codes

  • D56.1 vs. D56.0 (Alpha thalassemia) -
  • different globin chain affected. D56.1 vs. D56.5 (Hemoglobin E-beta thalassemia) -
  • D56.5 is for the specific compound heterozygous state with hemoglobin E. D56.1 vs. D57.40 (Sickle-cell thalassemia without crisis) -
  • D57.40 is for co-inheritance of sickle cell and thalassemia genes. D56.1 vs. D56.3 (Thalassemia minor) -
  • use D56.1 for beta thalassemia major and intermedia.

Child Codes

Code Hierarchy

Because D56.1 maps to an HCC category, the documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) before the diagnosis is used for risk adjustment.

D56.1 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 D56.1. 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 D56.1 in HCC Buddy

Open D56.1 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.