Unrelated Donor Umbilical Cord Blood Transplant Following Intrauterine Transfusions for Treatment of Alpha Thalassemia Major.

Abstract Homozygous α0-thalassemia (deletion of all 4 α-globin genes) results in Hb Bart’s (γ4 tetramers). The high oxygen affinity of Hgb Bart’s leads to severe hypoxia inutero with resulting profound edema (hydrops) and congestive heart failure. Almost universally fetal death occurs prior to diagnosis, therefore not allowing the opportunity for treatment. Advancement of maternal-fetal medicine and neonatal ultrasound has led …