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Erythrocyte Volume and Haemoglobin Concentration in Haemoglobin H Disease: Discrimination between the Two Genotypes

 

作者: Ahnond Bunyaratvej,   Punnee Butthep,   Suthat Fucharoen,   Daisy Saw,  

 

期刊: Acta Haematologica  (Karger Available online 1992)
卷期: Volume 87, issue 1-2  

页码: 1-5

 

ISSN:0001-5792

 

年代: 1992

 

DOI:10.1159/000204704

 

出版商: S. Karger AG

 

关键词: Cytometry;Erythrocyte;Haemoglobin Constant Spring;Haemoglobin H

 

数据来源: Karger

 

摘要:

Erythrocyte volume and haemoglobin concentration in individual red cells from 62 patients with Hb H disease: 37 with H genotype (α-thalassaemia 1/α-thalassaemia 2) and 25 H/CS genotype (α-thalassaemia 1/Hb Constant Spring) were measured using the H*l haematology analyser. All 25 cases with H/CS genotype, the more severe genotype, had microcytes (red cells with a volume smaller than 60 fl) less than 35% and hypochromic red cells (red cells with haemoglobin concentration less than 28 g/dl) more than 35%. A discriminant function, the ratio between the percentage of hypochromic red cells and the percentage of microcytes (Hypo/Micro), was proposed. Most of the H/CS patients (76%) had Hypo/Micro greater than 2.5 whereas those of H patients (82%) were below 2.5. Red cell volume histograms were also characteristically different between the two genotypes: the H/CS had a peak between 60 and 90 fl while the H genotype showed a peak at or very close to 60 fl, indicating a greater degree of microcytosis. Increased hypochromia with a slight decrease in cell size of H/CS red cells suggests that the poor degree of haemoglobinization has no linkage or very little role in disturbing the synthesis of membrane proteins and their assembly to the plasma membra

 

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