`D56.0` Alpha thalassemia

Billable ICD-10-CM code, FY2026.

Applicable to

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 (never code together)

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)

Applies from D56 Thalassemia

Excludes1 (never code together)

sickle-cell thalassemia (D57.4-)

Risk adjustment (HCC)

CMS-HCC V22: HCC 48 — Coagulation Defects and Other Specified Hematological Disorders
ESRD V24: HCC 48

MS-DRG index

Defines the logic of DRG(s) 791 in MDC 15.

Approximate ICD-9-CM (GEMs)

282.43 Alpha thalassemia

Code annotations containing back-references to D56.0

Back-references to D56.0

Type 1 Excludes: D56.3 Thalassemia minor ("alpha thalassemia (D56.0)")

Back-references to D56

Code First: M36.3 Arthropathy in other blood disorders ("thalassemia (D56.-)")
Code First: M90.5 Osteonecrosis in diseases classified elsewhere ("hemoglobinopathy (D50-D64)")
Type 1 Excludes: R71 Abnormality of red blood cells ("anemias (D50-D64)")

Diagnosis Index entries for D56.0

Disease, diseased › hemoglobin or Hb › Bart's
Disease, diseased › hemoglobin or Hb › H (Hb-H) (thalassemia)
Disease, diseased › hemoglobin or Hb › H (Hb-H) (thalassemia) › Constant Spring
Hb (abnormal) › Bart's disease
Hemoglobin › H Constant Spring
Hydrops › fetalis › due to › alpha thalassemia
Hydrops › newborn (idiopathic) › due to › alpha thalassemia
Thalassemia (anemia) (disease) › alpha (major) (severe) (triple gene defect)

Nearby codes

D56 Thalassemia
D56.0 Alpha thalassemia (this code)
D56.1 Beta thalassemia
D56.2 Delta-beta thalassemia
D56.3 Thalassemia minor
D56.4 Hereditary persistence of fetal hemoglobin [HPFH]
D56.5 Hemoglobin E-beta thalassemia