2027 ICD-10-CM Diagnosis Code D56.1Beta thalassemia
ICD-10-CM Codes›D50–D89›D55-D59›D56
- Billable — Valid for Submission
- Risk Adjusts — HCC 108
- Chronic Condition
D56.1 is a billable ICD-10-CM diagnosis code for beta thalassemia. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 791, 793, 811 through 812. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Hemolytic anemia.
For Medicare Advantage risk adjustment, D56.1 maps to CMS-HCC Category 108 (Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major) under the V28 model, adding a risk factor of about 0.146 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
D56.1 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.
Source: CMS Payment Year 2027 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 plus thalassemia normal hemoglobin A>2< type 1 silent
- Beta thalassemia
- Beta thalassemia intermedia
- Beta thalassemia major in mother complicating pregnancy
- Beta thalassemia X-linked thrombocytopenia syndrome
- Beta zero thalassemia
- Beta zero thalassemia deletion type
- Beta zero thalassemia non deletion type
- beta^+^ Thalassemia, normal Hb A>2<, type 2
- Dominant beta-thalassemia
- Hereditary persistence of fetal hemoglobin G gamma beta plus thalassemia
- Hereditary persistence of fetal hemoglobin thalassemia
- Homozygous beta thalassemia
- HPFH A gamma beta^+^ thalassemia
- Sickle cell beta plus thalassemia
- Sickle cell-beta-thalassemia
- Sickle cell-thalassemia disease
- Thalassemia intermedia
- Thalassemia major
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to D56.1: its own notes plus those printed at D56 and Chapter 3. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Beta thalassemia major
- Cooley's anemia
- Homozygous beta thalassemia
- Severe beta thalassemia
- Thalassemia intermedia
- Thalassemia major
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- autoimmune disease (systemic) NOS (M35.9)
- certain conditions originating in the perinatal period (P00-P96)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- human immunodeficiency virus [HIV] disease (B20)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name D56.1, its category, or a range that includes it.
Excludes1 3
These codes carry an Excludes1 note naming D56.1: they are not reported together with it, unless the two conditions are unrelated.
- D56.3 Thalassemia minorbeta thalassemia (D56.1)
- D56.5 Hemoglobin E-beta thalassemiabeta thalassemia (D56.1)
- Block P50-P61 Hemorrhagic and hematological disorders of newborn
Code First 1
These codes say to code first a condition named in the note, and D56.1 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound) D64.9
familial D56.1
of childhood D56.1
familial erythroblastic D56.1
Cooley's anemia D56.1
Thalassemia (anemia) (disease) D56.9
beta (severe) D56.1
homozygous D56.1
major D56.1
intermedia D56.1
major D56.1
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
beta-Thalassemia
a disorder characterized by reduced synthesis of the beta chains of hemoglobin. there is retardation of hemoglobin a synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, cooley's anemia, mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin a synthesis is absent.
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 D56.1 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About D56.1Overview
What is the ICD-10 code for beta thalassemia?
The ICD-10-CM code for beta thalassemia is D56.1 (sometimes written as D561). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is D56.1 (Thalassemia) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report beta thalassemia on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does D56.1 group to?
When beta thalassemia is the principal diagnosis on an inpatient stay, it groups to MS-DRG 793, 791, 812, 811, with relative weights from 0.9237 to 4.2192 depending on complications. Higher weights mean higher Medicare reimbursement.
Can D56.1 be reported with D56.3?
Not as a rule. The Excludes1 note on D56.1 lists "beta thalassemia minor (D56.3)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of D56.1?
Under the General Equivalence Mappings, beta thalassemia converts to ICD-9-CM 282.44 (beta thalassemia). The mapping is a direct match.
What HCC is D56.1?
D56.1 (beta thalassemia) maps to CMS-HCC Category 108 (Sickle Cell Disorders, Except Sickle Cell Anemia (Hb-SS) and Thalassemia Beta Zero; Beta Thalassemia Major), commonly written as HCC 108, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 48 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. It does not map to any RxHCC in the Part D prescription drug model.
Does D56.1 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, D56.1 adds a risk adjustment factor of about 0.146 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.098 to 0.416 depending on the payment segment). A more severe related category (HCC 107) supersedes it when both are reported. See the full factor table on the HCC 108 category page.
