Catalog

Hemoglobin Bart's fetalis syndrome

3A50.03

Link to WHO Definition

Hemoglobin Bart's fetalis syndrome – http://id.who.int/icd/entity/1859849042

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.

Parents