Hb Leiden-beta (0) thalassemia in a Chinese with severe hemolytic anemia

The first case of Hb Leiden (alpha2beta2 6 or 7 Glu---O)-beta (0) 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 beta chains when no blood transfusion was...

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Bibliographic Details
Published in:	American journal of hematology Vol. 2; no. 4; p. 335
Main Authors:	Lie-Injo, L E, Ganesan, J, Randhawa, Z I, Peterson, D, Kane, J P
Format:	Journal Article
Language:	English
Published:	United States 1977
Subjects:	Adolescent Anemia, Hemolytic - complications Asian Continental Ancestry Group Blood Transfusion Hemoglobinopathies - complications Hemoglobinopathies - genetics Hemoglobins, Abnormal - analysis Hepatomegaly - complications Humans Male Splenomegaly - complications Syndrome Thalassemia - complications Thalassemia - genetics
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Summary:	The first case of Hb Leiden (alpha2beta2 6 or 7 Glu---O)-beta (0) 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 beta chains when no blood transfusion was given. His mother was heterozygous for beta(0) thalassemia, and his father and brother had a 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.
ISSN:	0361-8609
DOI:	10.1002/ajh.2830020403