Hemoglobin E and pregnancy.

Hemoglobin E occurs in 30 million people, primarily Southeast Asians. Their resettlement within the US has dramatically increased the incidence of E hemoglobinopathies. A gravid Vietnamese woman with thalassemia major is reported herein. Her pregnancy was complicated by severe anemia, intrauterine growth retardation, and a paraspinal mass representing extramedullary hematopoiesis. The diagnosis of hemoglobin E/beta o-thalassemia was established when analysis of her hemoglobin showed 60% F, 40% E, and 0% A. The patient was transfused with packed red blood cells to maintain the maternal hematocrit at 30%. A term growth-retarded infant was delivered who had severe thrombocytopenia and an imperforate anus. The infant's thrombocytopenia responded only to infusion of maternal platelets. The differential diagnosis and expected hematologic manifestations of the various E hemoglobinopathies are detailed. Hematologic and obstetric guidelines for management during pregnancy are offered.