ALPHA-THALASSEMIAS

Citation
Jy. Peltier et al., ALPHA-THALASSEMIAS, Annales de biologie clinique, 52(5), 1994, pp. 321-331
Citations number
50
Categorie Soggetti
Medicine, Research & Experimental",Biology
ISSN journal
00033898
Volume
52
Issue
5
Year of publication
1994
Pages
321 - 331
Database
ISI
SICI code
0003-3898(1994)52:5<321:A>2.0.ZU;2-X
Abstract
alpha-Thalassaemias are probably the,Most common genetic disorder worl dwide. alpha-Thalassaemias are haemolytic anaemias resulting from inhe rited deficient synthesis of alpha-globin chains. In this paper the cl assification and nomenclature of alpha thalassaemias are developed. Pr ocedures to ensure the laboratory diagnosis are explained The heterozy gous carrier states for these disorders are, in most cases, not associ ated with any easily discernible change in the haemoglobin pattern, ex cept, sometimes, a reduced MCV in the blood picture. Heterozygotes alp ha-thalassaemia deletions are now detectable by the accurate PCR metho d Because of the high prevalence of these disorders in large segments of the world population, alpha-thalassaemia and haemogblobinopathies o ften occur in the same individual. The laboratory features of these in teractions and, particularly, the role of alpha-thalassaemia as a pote ntial modulator of sickling haemoglobinopathies are discussed.