ICD-11 classes
03 Diseases of the blood or blood-forming organs
Anaemias or other erythrocyte disorders
3A50 — Thalassaemias
3A50.0 — Alpha thalassaemia

ICD-11 3A50.0 — Alpha thalassaemia

Alpha-thalassemia is an inherited haemoglobinopathy characterised by impaired synthesis of alpha-globin chains leading to a variable clinical picture depending on the number of affected alleles, and encompassing the alpha thalassaemia trait, haemoglobin H disease (HbH) and Bart's hydrops fetalis.

The diagnosis includes nothing.

It excludes 1 item.

  • Hydrops fetalis due to haemolytic disease (KA85)

Diagnosis with code 3A50.0 contains 6 clarifying diagnoses:

  1. 3A50.00 — Mild alpha thalassaemia diseases
  2. 3A50.01 — Thalassaemic alpha-chain variants
  3. 3A50.02 — Haemoglobin H disease (– α/– – included)
  4. 3A50.03 — Homozygous or compound heterozygous alpha0 thalassaemia
  5. 3A50.0Y — Other specified alpha thalassaemia
  6. 3A50.0Z — Alpha thalassaemia, unspecified

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