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Hb leiden‐β° thalassemia in a chinese with severe hemolytic anemia
Author(s) -
LieInjo Luan Eng,
Randhawa Z. I.,
Ganesan J.,
Peterson D.,
Kane J. P.
Publication year - 1977
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830020403
Subject(s) - medicine , thalassemia , splenectomy , hemolytic anemia , brother , pediatrics , anemia , chinese family , compound heterozygosity , hemoglobin , blood transfusion , gastroenterology , mutation , genetics , gene , biology , spleen , sociology , anthropology
The first case of Hb Leiden (α 2 β 2 6 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.