alpha thalassemia-0 (homozygous alpha thalassemia)

Pathology

• predominant fetal hemoglobin is a hemoglobin Barts that has essentially no oxygen carrying capacity
• this causes oxygen starvation in the fetal tissues leading to prenatal lethality or early neonatal death (nonimmune hydrops fetalis)

Genetics

• 0 of 4 functional alpha globin genes

Laboratory

• complete blood count
  • anemia
  • microcytosis
• hemoglobin electrophoresis: hemoglobin Barts (100%)

More general terms

• alpha thalassemia

Additional terms

• hemoglobin Barts