title: Age and sex matched analysis of Hb Lepore trait in a new population in Spain.
abstract: A group of subjects with Hb Lepore trait has been found in the region between Extremadura and Toledo in Spain. Clinical, radiological and hematological studies were carried out on 81 cases from 23 families. Asthenia was the sole complaint in seven of forty cases. Abdominal echography showed no cholelithiasis in 16 children under 16 years. Hb Lepore mean was 10.81 +/- 1.97%, range 6.5 to 16.1%, Hb A2 levels were normal and Hb F values were high. Globin chain synthesis in reticulocytes showed a total alpha/beta ratio of 1.89 +/- 0.3. Hematological values from Hb Lepore trait subjects were analyzed according to age and sex and the data compared to beta thalassemia and delta-beta thalassemia cases of matched age and sex. Hb Lepore trait patients had a milder form of thalassemia minor than beta thalassemia patients, with higher levels of hemoglobin, MCV and MCH for all three groups: children under 13 years, males over 14 years and females over 14 years. Children and females with Hb Lepore had higher hemoglobin levels than those with delta-beta thalassemia minor, while no significant difference was found in males.
authors.affiliation: Servicio de Hematología, Hospital 12 de Octubre, Madrid, Spain.
authors.forename: F
authors.initials: F
authors.lastname: Gilsanz
authors.forename: J G
authors.initials: JG
authors.lastname: Vela
authors.forename: G M
authors.initials: GM
authors.lastname: Núñez
mesh_terms: D000293:Adolescent; D001853:Bone Marrow / Q000000981:diagnostic imaging; D002648:Child; D005260:Female; D006580:Genetic Carrier Screening; D006403:Hematologic Tests; D006455:Hemoglobins, Abnormal / Q000235:genetics*; D006801:Humans; D008297:Male; D011859:Radiography; D013030:Spain / Q000453:epidemiology; D013789:Thalassemia / Q000097:blood* / Q000000981:diagnostic imaging
chemical_list: D006455:Hemoglobins, Abnormal; C037659:hemoglobin Lepore