Hb leiden-β° thalassemia in a chinese with severe hemolytic anemia

Abstract
The first case of Hb Leiden (α2β26 or 7 Glu→0)‐β° thalassemia in a young patient with chronic severe hemolytic anemia, which improved after splenectomy, is described. His parents were Chinese. The patient's blood showed no Hb A or normal β chains when no blood transfusion was given. His mother was heterozygous for β° thalassemia, and his father and brother had the trait for the unstable Hb Leiden. The Hb Leiden level of the father was 22.6% and that of the brother was 19.3%. It is probable that the abnormal hemoglobin in this Chinese family resulted from an independent gene mutation, unrelated to the one found in 2 Caucasian families reported earlier.