Combination of three alpha-globin gene loci deletions and hemoglobin New York results in a severe hemoglobin H syndrome.

A chinese patient with hemoglobin (Hb) H and Hb New York is described. Unlike classical Hb H disease, the additional beta New York chain defect resulted in a more marked alpha/beta-globin chain imbalance in this patient with only one alpha-gene, accounting for her severe anemia (3.4-6.8 g/dl) during the steady state.