ICD-11 classes
03 Diseases of the blood or blood-forming organs
Anaemias or other erythrocyte disorders
3A50 — Thalassaemias
3A50.0 — Alpha thalassaemia
3A50.03 — Homozygous or compound heterozygous alpha0 thalassaemia

ICD-11 3A50.03 — Homozygous or compound heterozygous alpha0 thalassaemia

Hb Bart's hydrops fetalis is the most severe form of alpha-thalassemia and is almost always lethal. It is characterised by fetal onset of generalised oedema, pleural and pericardial effusions, and severe hypochromic anaemia.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.