title: Red blood cell phenotypes in alpha-thalassemias in the Spanish population.
abstract: OBJECTIVE
  alpha-thalassemia is very common on all thalassemic geographical regions. The present work aimed at analyzing the relationship between the degree of microcytosis and hematological parameters and the type of alpha-thalassemic mutation.
  
  
  METHODS
  Five hundred and thirty-six subjects with 4 kinds of alpha-thalassemia were examined using established techniques that determined all hematological parameters, and globin synthesis and molecular biological techniques to study the DNA of globin genes by Southern blotting.
  
  
  RESULTS
  Adult carriers of alpha (+)-thalassemia (-alpha/alpha alpha) present very few hematological alterations. In a statistical comparison with normal individuals (alpha alpha/alpha alpha), significant differences were found between the hemocytometric data and the MCV and MCH of heterozygous alpha + thalassemia and the heterozygous alpha zero or homozygous alpha + genotype. Hb H disease was detected in 15 patients, presenting a severe degree of anemia, a significant increase in RDW and globin chain synthesis with an alpha/beta ratio of 0.5 +/- 0.1.
  
  
  CONCLUSIONS
  These data provide reference values for geographical areas where alpha + thalassemia is common. These hematocytometric data, together with hemoglobin analysis, could be useful as a future reference data for new patients diagnosed with alpha-thalassemia.
authors.affiliation: Servicio de Hematología, Hospital Universitario San Carlos, Universidad Complutense, Madrid, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Villegas
authors.forename: A
authors.initials: A
authors.lastname: Porres
authors.forename: J
authors.initials: J
authors.lastname: Sánchez
authors.forename: F A
authors.initials: FA
authors.lastname: González
authors.forename: C
authors.initials: C
authors.lastname: Pérez-Clausell
authors.forename: M
authors.initials: M
authors.lastname: Martínez
authors.forename: M J
authors.initials: MJ
authors.lastname: Murga
authors.forename: J
authors.initials: J
authors.lastname: Cachá
authors.forename: M
authors.initials: M
authors.lastname: Lozano
authors.forename: I
authors.initials: I
authors.lastname: Fernández-Fuertes
authors.forename: A
authors.initials: A
authors.lastname: Del Arco
authors.forename: B
authors.initials: B
authors.lastname: Arrizabalaga
authors.forename: B
authors.initials: B
authors.lastname: Pérez de Mendiguren
authors.forename: I
authors.initials: I
authors.lastname: San Juan
authors.forename: R
authors.initials: R
authors.lastname: Saavedra
authors.forename: P
authors.initials: P
authors.lastname: Ricart
authors.forename: C
authors.initials: C
authors.lastname: Sainz
authors.forename: J L
authors.initials: JL
authors.lastname: Guerra
authors.forename: J A
authors.initials: JA
authors.lastname: Muñoz
authors.forename: C
authors.initials: C
authors.lastname: Lago
authors.forename: V M
authors.initials: VM
authors.lastname: Ansó
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D004909:Erythrocyte Indices; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D005838:Genotype; D006400:Hematocrit; D006447:Hemoglobin H / Q000235:genetics; D006801:Humans; D008297:Male; D010641:Phenotype; D017701:Reticulocyte Count; D012737:Sex Factors; D013030:Spain; D017085:alpha-Thalassemia / Q000097:blood / Q000145:classification / Q000235:genetics*
chemical_list: D006447:Hemoglobin H