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Peripheral blood smear and hemoglobin electrophoresis of unsuspected hemoglobin Bart's hydrops fetalis in a newborn

Peripheral blood smear was received on a preterm male delivered at 29week gestation by emergency cesarean section for fetal distress. Pulmonary and cardiac malformations had been noted on obstetric ultrasound. Complete blood count (CBC) on the neonate was significant for severe anemia (hemoglobin 5 g/dL). The blood smear showed marked hypochromia, anisopoikilocytosis, and nucleated RBCs (Figure 1). Hemoglobin F/A/A2 were absent on HPLC analysis of newborn specimen. Both parents are Vietnamese, and two teenaged sisters are healthy. Maternal CBC showed mild microcytic anemia (hemoglobin 11 g/dL, MCV 75). Prenatal hemoglobin HPLC had been reported as Hb E trait. Hemoglobin analysis of maternal and neonatal specimens by capillary electrophoresis is shown in Figure 2A and 2B. Maternal specimen has 79% Hb A (position 150), 4% Hb A2 (at position 243), 16% variant hemoglobin in the Hb E zone (anodal to Hb A2), and 0.8% Hb F. In uncomplicated E trait, Hb E is 30%35% of total hemoglobin. Concurrent alphathalassemia or iron deficiency will lower the variant hemoglobin percentage and the MCV. Hb E is typically 25%30% in Hb E/α+thalassemia trait (deletion or inactivation of one αglobin gene or α/αα ).1 Low maternal Hb E of 16% is consistent with compound heterozygosity for α0thalassemia trait (2gene deletion or /αα) and E trait. Paternal CBC was not available but microcytosis associated with α0thalassemia trait would be expected. The infant has 84% Hemoglobin Bart's (γ4) and 13% variant in the Hb F zone, which represents embryonic Hemoglobin Portland (ζ2γ2). This pattern is consistent with homozygous α0 thalassemia (/).13 Other minor peaks3 are presumably epsilon 4 (position 120), and Hemoglobin Gower1 (ζ2ε2 ) at position 160. Hemoglobin Bart's hydrops fetalis syndrome is lethal in utero or soon after birth and is associated with an average hemoglobin of 6.3 g/dL, congenital malformations, and maternal complications such as preeclampsia.4 Screening for alphathalassemia has been combined with betathalassemia screening programs in regions of the world where both are prevalent, such as Hong Kong.5 Alphathalassemia trait is electrophoretically silent unlike betathalassemia trait, which produces an increase in Hb A2. As a result, alphathalassemia trait is more easily overlooked on prenatal hemoglobin electrophoresis. The key point in this case was the subtle electrophoretic signature of low maternal Hb E percentage, much lower than the typical 30%35% seen in uncomplicated Hb E trait. In practice, this finding warrants further investigation which includes iron studies and paternal CBC, with referral for DNA analysis as necessary.