D56.0

Billable
ChapterD50–D89
Ranksubcategory
Official Description

Alpha thalassemia

Metadata & Insights
  • •2026 ICD-10-CM Edition - Effective October 1, 2025.
  • •Billable/specific code (valid for reimbursement).
  • •Chapter 3: Diseases of the Blood and Blood-Forming Organs (D50-D89).

Instructional Notes

Includes
  • Alpha thalassemia major
  • Hemoglobin H Constant Spring
  • Hemoglobin H disease
  • Hydrops fetalis due to alpha thalassemia
  • Severe alpha thalassemia
  • Triple gene defect alpha thalassemia
Excludes1(Not coded here)
  • alpha thalassemia trait or minor (D56.3)
  • asymptomatic alpha thalassemia (D56.3)
  • hydrops fetalis due to isoimmunization (P56.0)
  • hydrops fetalis not due to immune hemolysis (P83.2)
Use Additional Code
  • code, if applicable, for hydrops fetalis due to alpha thalassemia (P56.99)
General instructions are available for the parent sections:
View CH3 NotesView D56 Notes

Clinical Hierarchy

CH3Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
D55-D59Hemolytic anemias (D55-D59)
D56Thalassemia
D56.0Alpha thalassemia

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to D56.0:

categoryD56
Thalassemia
View context instructions
chapterCH3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to D56.0. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

8 raw paths
Disease, diseased3 unique routes
hemoglobin or Hb(3 variants)
Bart's→D56.0
H (Hb-H) (thalassemia)→D56.0(2 variants)
Constant Spring→D56.0
Hb (abnormal)1 unique routes
Bart's disease→D56.0
Hemoglobin1 unique routes
H Constant Spring→D56.0
Hydrops2 unique routes
fetalis
due to
alpha thalassemia→D56.0
newborn (idiopathic)
due to
alpha thalassemia→D56.0
Thalassemia (anemia) (disease)1 unique routes
alpha (major) (severe) (triple gene defect)→D56.0

MS-DRG Classification

2026 v43.1

This code is grouped within the following Diagnosis Related Group(s) for clinical reimbursement:

DRG 791
Prematurity with Major Problems
DRG 793
Full Term Neonate with Major Problems
DRG 811
Red Blood Cell Disorders with MCC
DRG 812
Red Blood Cell Disorders without MCC

Cross Reference

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