3A50.03 Hemoglobin Bart's fetalis syndrome
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01
Hb Bart's hydrops fetalis (HBFS) 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. HBFS can be caused by either homozygous or compound heterozygous alpha0 (alpha zero) thalassaemia, resulting in zero functional alpha haemoglobin genes.
synonyms
- Hemoglobin Bart's fetalis syndrome
- BHFS - [Bart's hydrops fetalis syndrome]
- HBHF - [Hb Bart's hydrops fetalis]
- Haemoglobin Bart's disease
- Hb Bart's hydrops fetalis
- Hb Bart's hydrops fetalis syndrome
- alpha thalassaemia hydrops fetalis
- alpha-thalassemia major
- haemoglobin Bart hydrops fetalis
- homozygous alpha0-thalassemia
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