3A50.03

ICD-11 MMS 2026-01

Hemoglobin Bart's fetalis syndrome

Description

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.

Index terms

  • Hemoglobin Bart's fetalis syndrome
  • BHFS - [Bart's hydrops fetalis syndrome]
  • HBHF - [Hb Bart's hydrops fetalis]
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  • 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

Exclusions from above levels

  • KA85 – Hydrops fetalis due to haemolytic disease
  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth or the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3A50.00 – Mild alpha thalassaemia diseases
  • 3A50.01 – Thalassaemic alpha-chain variants
  • 3A50.02 – Haemoglobin H disease (– α/– – included)
  • 3A50.0Y – Other specified alpha thalassaemia
  • 3A50.0Z – Alpha thalassaemia, unspecified

Foundation entity

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

Part of: 3A50.0: Alpha thalassaemia › 3A50: Thalassaemias › 03: Diseases of the blood or blood-forming organs