Hydrops fetalis syndrome

Also known as: Hb Bart's hydrops fetalis syndrome

Severe alpha thalassemia causing fetal hydrops.

Medical specialty: Hematology
Subspecialty: Hematology / Hemoglobinopathies
Sex & age distribution: Male: affected; Female: affected; Children: yes, prenatal
Gene(s) involved: HBA1, HBA2

Etiology & mechanism

Deletion of all four alpha globin genes.

Gene(s) involved (2)

HBA1HBA2

Symptoms & signs (4)

fetal hydropshepatosplenomegalysevere anemiastillbirth

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